PATHOLOGY: Implications for the Physical Therapist

MEDICAL MANAGEMENT

DIAGNOSIS.

Diagnosis depends on the previous history, clinical presentation, and, if needed, skin biopsy to identify psoriatic changes in skin or rule out other causes for the lesions. Typically, the serum uric acid level is elevated because of accelerated nucleic acid degradation but without the corresponding gout usually associated with increased uric acid levels. Psoriasis must be distinguished from eczema, seborrheic dermatitis, and lichenlike papules.

TREATMENT.

In the absence of a cure, the goal of treatment is to maximize remission and lessen outbreaks. Psoriasis therapy is highly individualized and often determined by trial and error because different people respond to different treatments. Psoriasis does not spread, and early treatment does not prevent the condition from progressing.

New options exist that adequately suppress the disease process and help provide better control of the psoriasis through the use of a combination of therapies. Various forms of local or systemic treatment routinely offered fall into five general categories: (1) topical preparations, (2) phototherapy, (3) antimetabolites, (4) oral retinoid therapy, and (5) immunosuppressants. New biologic systemic drugs for moderate to severe psoriasis are now available.⁸⁰

Identifying individuals who would be good candidates for systemic therapy is under investigation; use of a formal diagnostic tool called the Koo-Menter Psoriasis Instrument (KMPI) has been advocated in making this treatment decision.⁴¹

Topical treatment of psoriasis is usually the first line of therapy, and therapeutic agents include corticosteroids; synthetic vitamin D₃; vitamin A analogues (retinoids); occlusive ointments (e.g., petroleum jelly, salicylic acid preparations, urea-containing topical ointments); oatmeal baths and emollients to relieve pruritus; and occasionally tar preparations.

Corticosteroids are the most commonly prescribed therapy for psoriasis but should be used sparingly because of the incidence of side effects, which have increased with the use of the superpotent fluorinated preparations. Only weak preparations, such as 0.5% or 1.0% hydrocortisone, should be used on the face, perineum, or other sensitive areas (e.g., the flexor surfaces of the arms, abdomen).

The major concerns with all corticosteroid preparations are dermal atrophy, skin fragility, fast relapse times, and, in rare cases, adrenal suppression resulting from systemic absorption. See also the section on Corticosteroids in Chapter 5.

Crude coal tar, one of the oldest remedies for psoriasis, is assumed to work by an antimitotic effect (helps retard rapid cell production). This treatment consists of the daily application of 2% to 5% crude coal tar combined with a tar bath and UV light. The disadvantages of this treatment are the extended time commitments required by the client and the associated mess. More recently, the use of liquid carbonis detergens (LCD) has replaced the use of crude coal tar.

Exposure to UV light (phototherapy), such as UVB or natural sunlight, also helps retard rapid cell production. Widespread involvement may improve with whole-body irradiation with UV light. PUVA refers to the combination of an orally administered photosensitizing drug plus exposure to 1 to 1½ hours of UVA radiation. It is more effective for the thick plaque type of psoriasis, pustular psoriasis, and generalized erythroderma.

PUVA also has its disadvantages as a treatment option, including premature skin aging, increased risk of nonmelanoma skin and other cancers, and premature cataract formation. This type of therapy is contraindicated in pregnancy and for anyone with abnormal moles or otherwise at risk for skin cancer.

Methotrexate, originally an anticancer drug, affects DNA synthesis and inhibits reproduction in rapidly growing cells, such as the prolific keratinocytes in psoriasis. Methotrexate also has an immunosuppressant effect, tempering the inflammatory response. Cyclosporine (Neoral), an immunosuppressant most often used to prevent organ transplant rejection, has also been approved as a psoriasis treatment. These pharmaceuticals have potentially serious side effects and must be monitored closely.

A treatment strategy called sequential therapy involving a deliberate sequence to optimize therapeutic outcome is being explored for those who require systemic therapy without methotrexate. The rationale for this strategy in psoriasis is that it is a chronic disease requiring long-term maintenance therapy as well as quick relief of symptoms and that some medical interventions are better suited for rapid clearance whereas others are more appropriate for long-term care. In sequential therapy, an acute exacerbation of psoriasis is brought under control promptly with the use of cyclosporine followed by phototherapy and then acitretin is administered in a maintenance phase.^78,79

To minimize side effects and maximize efficacy of rapidly clearing lesions and maintaining remission, topical sequential therapy is widely used. A class I corticosteroid and calcipotriene are applied in three different phases—the clearance, transition, and maintenance phases.⁸¹

PROGNOSIS.

Psoriasis usually recurs at intervals and lasts for increasingly longer periods, but treatment advances bring relief during flare-ups in approximately 85% to 90% of cases. Spontaneous cure is uncommon, and the risk of infection is high because of the greater than normal amounts of staphylococci present on psoriatic plaques.

People with psoriasis who are HIV positive are at high risk of infection from self-inoculation. As many as 20% of clients who develop psoriatic arthritis may sustain early and severe joint damage with accompanying deformity and disability.

Finally, psoriasis treatment involving PUVA has been shown to contribute to an increased risk of skin cancer decades after the treatment has stopped.¹⁴⁴ New research shows that it may be possible to reduce the risk of skin cancer from PUVA with meditation and stress reduction techniques that reduced healing time to half, thus reducing UV exposure.⁷³

10-11 SPECIAL IMPLICATIONS FOR THE THERAPIST

Psoriasis

PREFERRED PRACTICE PATTERNS

7A:

Primary Prevention/Risk Reduction for Integumentary Disorders

7B:

Impaired Integumentary Integrity Associated with Superficial Skin Involvement

Physical therapy and occupational therapy are key components in the treatment of moderate to severe psoriasis, with desired outcomes based on minimizing functional limitations.^*

Client instruction and direct intervention to provide skin care should emphasize the following: (1) steroid cream application must be in a thin film, rubbed gently into the skin until all the cream disappears; (2) all topical medications, especially those containing anthralin and tar, should be applied with a downward motion to avoid rubbing them into the hair follicles causing inflammation (folliculitis); (3) medication should be applied only to the affected lesions, avoiding contact with normal surrounding skin; and (4) gloves must be worn when applying the cream since anthralin stains and injures the skin.

After application, the client must dust himself or herself with powder to prevent anthralin from rubbing off on the clothes. Mineral oil followed by soap and water can be used to remove the anthralin; the skin should never be rubbed vigorously, but a soft brush can be used to remove the scales.

Any side effects, especially allergic reactions to anthralin, atrophy and acne from steroids, and burning, itching, and nausea, must be reported to the physician immediately. Squamous cell epithelioma may develop from PUVA. Cytotoxins from methotrexate therapy may cause hepatic or bone marrow toxicity; methotrexate may be teratogenic (harmful to fetal development) and should not be prescribed for women who are pregnant, trying to become pregnant, or breast feeding.

Other immunosuppressants, when used over a long period, have an accumulative effect and therefore the potential to cause serious side effects, such as poor wound healing, high blood pressure, kidney damage, and many other complications (see the section on Immunosuppressants in Chapter 5).

Relaxation techniques and stress management are valuable tools whose use should be encouraged on a daily basis but especially during periods of exacerbation.

Psoriatic Arthritis

Clinically, psoriatic arthritis differs from rheumatoid arthritis in the more frequent involvement of the distal interphalangeal joints, asymmetric distribution of affected joints, presence of spondyloarthropathy (including the presence of both sacroiliitis and spondylitis), and characteristic extraarticular features (e.g., psoriatic skin lesions, iritis, mouth ulcers, urethritis, colitis, aortic valve disease).

Joints are less tender in psoriatic arthritis, which may lead to underestimation of the degree of inflammation. Pain and stiffness of inflamed joints are usually increased by prolonged immobility and alleviated by physical activity. Evidence of inflammation is pain on stressing the joint, tenderness at the joint line, and the presence of effusions.

The increasing use of nuclear magnetic resonance imaging (MRI) techniques, with their ability to delineate cartilage and ligamentous structures and to identify edema, is providing a radical improvement in ascertainment of musculoskeletal abnormalities associated with this disease. It is expected that in the decade to come new information about this aspect of the disease will offer greater information and improved treatment regimens.¹⁶⁰

Psychologic Considerations

Psoriasis can result in psychologic problems because the skin lesions may cause the person to feel contagious and untouchable. In addition, ongoing treatment may not work, and the smell of some topical preparations and the stain may add to the psychologic reaction. Assure the client that psoriasis is not contagious. Flare-ups can be controlled with treatment, and stress control can help prevent recurrences. Relaxation techniques, group counseling, stress management, and medications to treat depression or anxiety may be suggested.

^*For an interesting and comprehensive article on establishing a practical and effective psoriasis treatment center including phototherapy, debridement, and whirlpool therapy, see reference 6.

Lupus Erythematosus

Lupus erythematosus is a chronic inflammatory disorder of the connective tissues. It appears in several forms, including cutaneous lupus erythematosus primarily affecting the skin and systemic lupus erythematosus (SLE), which affects multiple organ systems (including the skin) with considerably more morbidity and associated mortality (see complete discussion in Chapter 7). Lupus is the Latin word for “wolf,” referring to the belief in the 1800s that the skin erosion of this disease was caused by a wolf bite. The characteristic rash of lupus is red, hence the term erythematosus.

Cutaneous Lupus Erythematosus

Overview and Incidence.: The subsets of lupus erythematosus (LE) involving the skin include chronic cutaneous LE, acute cutaneous LE, and subacute cutaneous LE. Only the skin-related components of LE are discussed in this chapter. See also the section on Systemic Lupus Erythematosus in Chapter 7.

Chronic cutaneous LE, formerly known as discoid lupus, is marked by chronic skin eruptions on sun-exposed skin that can lead to scarring and permanent disfigurement if left untreated. Usually a systemic disorder does not develop, but in 5% to 10% of cases SLE does develop later; conversely, discoid lesions occur in 20% of people with SLE.¹³⁴ It is estimated that approximately 60% of persons with chronic cutaneous LE are women in their late twenties or older. The disease is rare in children.

Acute cutaneous LE occurs in 30% to 50% of clients who have SLE and includes malar erythema, widespread erythema, and bullous lesions. Association with systemic disease is highest in acute cutaneous LE, with virtually all clients meeting the American College of Rheumatology criteria for SLE (see Chapter 7).

Etiologic and Risk Factors and Pathogenesis.: The exact cause of cutaneous LE is unknown, but evidence suggests an autoimmune defect. There appear to be interrelated immunologic, environmental, hormonal, and genetic factors involved. Smoking is considered a risk factor for the development of the discoid lesions associated with chronic cutaneous LE and for resistance to treatment with antimalarial agents in this subgroup.^46,71

Just how the sun causes skin rash flare-ups remains unknown. One theory is that the DNA of people with lupus, when exposed to sunlight, becomes more antigenic (able to induce a specific immune response). This antigenicity causes accelerated antigen-antibody reactions and thus more deposition of immune complexes in the skin at the dermal-epidermal junction. The photosensitivity is most commonly associated with LE and not other rheumatologic diseases.

Clinical Manifestations.: Discoid lesions (chronic cutaneous LE) can develop from the rash typically seen in lupus and become raised, red, smooth plaques with follicular plugging and central atrophy. The raised edges and sunken centers give them a coinlike appearance (Fig. 10-20). Although these lesions can appear anywhere on the body, they usually erupt on the face, scalp, ears, neck, and arms or any part of the body that is exposed to sunlight. Lesions more typical of systemic lupus discussed in Chapter 7 are shown in Fig. 10-21.

Figure 10-20 Discoid lupus erythematosus. Skin changes associated with discoid lupus erythematosus can present in a variety of ways. A, Hypertrophic discoid lupus erythematosus with prominent adherent scale. B, Round or oval cutaneous lesions can occur on the face or other parts of the body. C, Round or oval cutaneous lesions as they appear on a dark-skinned individual. (From Callen JP, Greer K, Paller A, Swinyer L: Color atlas of dermatology, ed 2, Philadelphia, 2000, Saunders.)

Figure 10-21 The lesions here are typical of systemic lupus found on the lower extremities. They are ulcerated, punched-out wounds with necrotic bases. Discoid lupus lesions (Fig. 10-20) are usually found on the face and scalp, and are raised, flat, coin-shaped wounds. (Courtesy Harriett B. Loehne, PT, DPT, CWS, FCCWS, Archbold Center for Wound Management, Thomasville, GA, 2006. Used with permission.)

Hair tends to become brittle, and scalp lesions can cause localized alopecia (bald patches). Facial plaques sometimes assume the classic butterfly pattern with lesions appearing on the cheeks and the bridge of the nose. The rash may vary in severity from a sunburned appearance to discoid (plaquelike) lesions. These lesions can occur in the absence of other lupus-related symptoms and tend to leave hypopigmented and hyperpigmented scars that can become a cosmetic concern.

The most recognized skin manifestation of SLE (acute cutaneous LE) is the classic butterfly rash over the nose, cheeks, and forehead (Fig. 10-22) commonly precipitated by exposure to sunlight (UV rays). This classic rash over the nose and cheeks occurs in a large percentage of affected people, but rash can occur on the scalp, neck, upper chest, shoulders, extensor surface of the arms, and dorsum of the hands. These rashes begin abruptly and last from hours to days. They may be precipitated by sun exposure and often coincide with a flare of systemic disease.¹³⁴

Other skin manifestations may point to the presence of vasculitis (inflammation of cutaneous blood vessels) leading to infarctive lesions in the digits (see Fig. 27-14), necrotic leg ulcers, or digital gangrene.

Acute cutaneous LE is usually accompanied by other symptoms of SLE, commonly including malaise, overwhelming fatigue, arthralgia, fever, arthritis, anemia, hair loss, Raynaud’s phenomenon, and urologic symptoms associated with kidney involvement.

MEDICAL MANAGEMENT

DIAGNOSIS AND TREATMENT.

The client history and appearance of the rash itself are diagnostic. Skin biopsy of the discoid lesions may be performed. The client must report any changes in the lesions to the attending physician. Drug treatment consists of topical, intralesional, or systemic medication.

Potential side effects of systemic therapy (antimalarial agents) for chronic cutaneous LE include diarrhea, nausea, myopathy, cardiomyopathy, and anemia. The lesions resolve spontaneously in 20% to 40% of affected individuals or may cause hypopigmentation or hyperpigmentation, atrophy, and scarring. Discoid lesions are not life-threatening (unless accompanied by complications of SLE) but are associated with psychologic distress and altered quality of life.

Skin lesions require topical treatment, maintaining an optimal wound environment (moist enough to allow tissue healing but not swamplike) while preventing further deterioration or infection. Most often, topical corticosteroid creams are used. The disease process can cause loss of skin integrity and subsequent loss of function.

Clients with any form of cutaneous lupus should avoid prolonged exposure to the sun, fluorescent lighting, or reflected sunlight. They are encouraged to wear protective clothing, use sun-screening agents, and avoid engaging in outdoor activities during periods of intense sunlight (see Box 10-4).

PROGNOSIS.

The survival rate has improved dramatically in recent years, although death can occur from renal failure when there is kidney involvement causing progressive changes in the glomeruli; cardiac involvement with deposition of immune complexes in the coronary vessels, myocardium, and pericardium; or cerebral infarct.

10-12 SPECIAL IMPLICATIONS FOR THE THERAPIST

Cutaneous Lupus Erythematosus

PREFERRED PRACTICE PATTERNS

4A:

Primary Prevention/Risk Reduction for Skeletal Demineralization

4B:

Impaired Posture

6B:

Impaired Aerobic Capacity/Endurance Associated with Deconditioning

7A:

Primary Prevention/Risk Reduction for Integumentary Disorders

Clients with lupus erythema with skin involvement require careful assessment, supportive measures, and emotional support. Skin lesions should be checked thoroughly at each visit. The client should be urged to get plenty of rest, follow energy conservation guidelines, and practice good nutrition.

The therapist can be instrumental in teaching and assisting with skin care and prevention of skin breakdown, range-of-motion (ROM) exercises, prevention of orthopedic deformities, ergonomic and postural training, and relief of joint pain associated with SLE. Persons with LE exposed to the long-term effects of corticosteroids should be followed carefully. See specific side effects and the section on Corticosteroids: Special Implications for the Therapist in Chapter 5. See also the section on SLE: Special Implications for the Therapist in Chapter 7.

Systemic Sclerosis

Definition and Overview

Systemic sclerosis (SSc, progressive systemic sclerosis [PSS], scleroderma) is a diffuse connective tissue disease that causes fibrosis of the skin, joints, blood vessels, and internal organs. SSc is a chronic disease, lasting for months, years, or a lifetime, and is classified according to the degree and extent of skin thickening.

The presence of a distinctive, widespread vascular lesion characterized by endothelial abnormalities as well as by proliferative reaction of the vascular intima was a significant factor in changing terminology from scleroderma to systemic sclerosis. General clinical vernacular still refers to this condition as scleroderma, although that term simply refers to thickening or hardening of the skin.

There are two distinct subtypes: systemic scleroderma and localized scleroderma (Box 10-12). Systemic scleroderma can take one of three forms: limited (lSSc), diffuse (dSSc), and an overlap form with either diffuse or limited skin thickening.

Box 10-12 CLASSIFICATION OF SCLERODERMA

Systemic Sclerosis

Limited (75%-80%)

• Symmetric skin thickening

• Restricted to distal extremities and face

• Slow progression of skin changes

• Late development of visceral involvement

• CREST syndrome

• Relatively good prognosis (≥70% survival at 10 years)

Diffuse (15%-20%)

• Symmetric skin thickening

• Widespread, affecting distal and proximal extremities, face, trunk

• Rapid progression of skin changes

• Early appearance of visceral involvement (GI tract, lungs, heart, kidneys)

• Overall poor prognosis (40%-60% survival at 10 years)

Overlap (5%-10%)

• Either diffuse or limited skin thickening

• Associated with one or more connective tissue diseases (e.g., systemic lupus erythematosus, dermatomyositis)

Localized

Morphea

• Single or multiple plaques of skin fibrosis without systemic disease

Linear

• Single or multiple fibrotic bands involving skin and deeper tissues

CREST, Calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia; GI, gastrointestinal.

Limited cutaneous SSc was previously known as the CREST syndrome from its manifestations (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia). Persons with this form of SSc have a much lower incidence of serious internal organ involvement, although pulmonary hypertension and esophageal disease are not uncommon. Skin tightness is limited to the hands and face (excluding the trunk).

Although the diffuse form is less common than the limited form, it is by far the more debilitating because of the more frequent renal and pulmonary involvement. Some measurable degree of heart, lung, or kidney involvement, or any combination of these, can be found in the majority of people with SSc.

Diffuse scleroderma is characterized by involvement of all body parts, including the skin. In most people, this involvement tends to progress slowly, if at all, but if involvement is to become severe, it tends to do so early, within the first 5 years. The severity of the disease depends on the number of organs affected and the extent of the effect.

Localized scleroderma affects primarily the skin in one or many different areas without visceral organ involvement and is therefore a benign form of this disease. Localized scleroderma should not be confused with limited cutaneous scleroderma. The latter is a form of systemic rather than localized disease.

There is further differentiation of localized scleroderma: morphea is characterized by hard, oval-shaped patches on the skin, generally on the trunk. These patches are usually white with a purple ring around them. Linear refers to the bandlike lesions that occur in the areas of the arms, legs, and forehead. The bones and muscles beneath these areas may also be affected. Ultimately, ROM and a child’s growth are greatly affected. Linear scleroderma often occurs in childhood.

Other forms include chemically induced localized scleroderma, eosinophilic myalgia syndrome (previously associated with ingestion of L-tryptophan), toxic oil syndrome (associated with ingestion of contaminated rapeseed oil), and graft-versus-host disease.

Incidence

The annual incidence of SSc based on epidemiologic studies of hospital records and death certificates is 10 to 20 cases per 1 million, affecting approximately 400,000 Americans. Until the 1980s, SSc was considered a rare (1 per 2000 individuals) disease, but studies at that time reported a prevalence as much as five times greater than the highest prevalence rate previously reported. Some evidence suggests continued rising incidence rates among adults as well as children, but whether this reflects worldwide incidence or merely regional differences remains unknown.^14,40

SSc affects women two to three times more often than men, with the female/male ratio peaking at 15: 1 during the childbearing years. Preliminary studies show that fetal cells persist in maternal blood for as long as 27 years postpartum even if the pregnancy does not go to full term. This phenomenon, referred to as fetomaternal cell trafficking, may provide an explanation for the increased prevalence of autoimmune disorders such as SSc in adult women following childbearing.¹²

Etiologic and Risk Factors

The cause of scleroderma is reportedly unknown. However, several groups suggest that scientific evidence accumulated over the last 50 years strongly points to SSc as an acquired disease triggered by bacteria (mycoplasma).^22,86 It has also been suggested that an autoimmune mechanism is the underlying cause, because specific autoantibodies occur in the sera of these clients. Other possible triggers suggested include cytomegalovirus (CMV; increased levels of anti-CMV antibodies present in scleroderma) or immune reactions to viral or environmental factors.⁷⁴

The potential role of placental transfer of fetal cells in the pathogenesis of autoimmune diseases has been mentioned (see Incidence). In childbearing women with scleroderma, fetal cell–derived DNA is detected more frequently in the peripheral blood than in controls. This finding of a limited number of fetal cells in maternal tissues leading to microchimerism (see explanation of chimerism in Chapter 21) has been proposed to have a role in the induction of scleroderma.¹⁴⁸

The occasional onset after trauma suggests the possibility of trophoneurosis (a trophic disorder consequent to disease or injury to nerves). The onset of scleroderma immediately following a severe emotional shock is in some cases a manifestation and result of a psychosomatic disturbance that causes vascular spasm.

Chemicals, especially from occupational exposure to silica, vinyl chloride, or various organic solvents (whether through direct contact or by inhalation), may also induce scleroderma-like changes. Exposure to organic solvents such as benzene and trichloroethane, chemicals common in paint thinners, stains, epoxy resins, and degreasers, associated with recreational hobby or occupation causes the body to produce an antibody called Scl-70 associated with scleroderma-like illnesses.^105,106 The toxic oil syndrome and eosinophilia-myalgia syndrome are best-known examples of chemically induced localized forms of scleroderma.³³

Pathogenesis

Widespread small-vessel vasculopathy and fibrosis set SSc apart from other connective tissue diseases. The relentless deposition of extracellular matrix (collagen) in the intima of blood vessels, the pericapillary space, and the interstitium of the skin is distinctive for SSc and distinguishes it from other autoimmune disorders.

Endothelial injury, obliterative microvascular lesions, and increased vascular wall thickness preferentially affecting small arteries, arterioles, and capillaries are present in all involved organs. Autonomic nerve dysfunction (parasympathetic impairment and marked sympathetic overactivity) seems to be linked to the development of microvascular, cardiac, and GI alterations.⁵⁰

The vascular pathologic condition is characterized by altered vascular function with increased vasospasm, reduced vasodilatory capacity, and increased adhesiveness of the blood vessels to platelets and lymphocytes. The connection between the vascular pathologic condition and development of tissue fibrosis remains unknown, but it is hypothesized that SSc modifies the activity of both the endothelium and the peripheral nervous system, eventually leading to the clinical manifestations of this condition.⁵¹ The extent of injury and dysfunction is reflected by changes in the circulating levels of vascular markers.⁷⁴

Clinical Manifestations

The three stages in the clinical development of scleroderma are the edematous stage, the sclerotic stage, and the atrophic stage. In the edematous stage, bilateral nonpitting edema is present in the fingers and hands and, rarely, in the feet. The edema can progress to the forearms, arms, upper chest, abdomen, back, and face. After a few weeks to several months, edema is replaced by thick, hard skin.

The replacement of edema takes place in the sclerotic stage, when the skin becomes tight, smooth, and waxy and seems bound down to underlying structures. Accompanying changes include a loss of normal skin folds, decreased flexibility, and skin hyperpigmentation and hypopigmentation.

The skin changes may stabilize for periods (years) and may then either progress to the third stage or soften and return to normal. Actual atrophy of skin may occur, particularly over joints at sites of flexion contractures, such as the proximal interphalangeal joints and the elbows. Such thinning of the skin contributes to the development of ulcerations at these sites. Softening and return to normal of the skin may occur to some extent. Improvement typically begins centrally, so that the last areas to become classically involved are the first to show regression.

Not all people pass through all the stages. Subcutaneous calcification (calcinosis) is a late-developing complication that is considerably more frequent in lSSc. Sites of trauma are often affected, such as the fingers, forearms, elbows, and knees. These calcifications vary in size from tiny deposits to large masses ulcerating the overlying skin.

Raynaud’s Phenomenon.: Scleroderma affects everyone in a different fashion. Each previously mentioned form affects the body in different ways (Box 10-13). Raynaud’s phenomenon is very often the first manifestation of SSc, preceding the onset of all the other signs and symptoms of the disease by months or years.⁵¹ It appears almost universally in lSSc and in approximately 75% of cases of dSSc.

Box 10-13 CHARACTERISTICS LIKELY TO BE SEEN IN CLIENTS WITH SYSTEMIC SCLEROSIS EARLY AND LATE IN THE DISEASE COURSE

Early (≤5 Years)

Limited Disease

• Rapidly progressive

• Renal crisis (5%)

• Interstitial lung disease (severe in 10%-15%)

• Slowly progressive

• Raynaud’s phenomenon

• Cutaneous ulceration

• Esophageal dysmotility

Diffuse Disease

• Rapidly progressive

• Skin thickening

• Heart involvement (severe in 10%-15%)

• Interstitial lung disease (severe in 15%)

• Renal crisis (15%-20%)

• Contractures, joint pain

• Cutaneous ulcerations

• Esophageal dysmotility

• Gastrointestinal complications

Late (>5 Years)

Limited Disease

• Slowly progressive

• Raynaud’s phenomenon

• Cutaneous ulcerations

• Esophageal dysmotility

• Gastrointestinal complications

• Very late

• Pulmonary artery hypertension

• Biliary cirrhosis

Diffuse Disease

• Improvement

• Skin thickening

• Musculoskeletal pain

• Slowly progressive

• Heart, lung, kidney involvement

• Raynaud’s phenomenon

• Esophageal dysmotility

• Gastrointestinal complications

Modified from Clements PJ: Systemic sclerosis: natural history and management strategies, J Musculoskel Med 11(11):43-50, 1994.

Raynaud’s phenomenon is characterized by sudden blanching, cyanosis, and erythema of the fingers and toes as the walls of the blood vessels that supply the hands and feet become narrowed, making it difficult for the blood to pass through. Closure of the muscular digital arteries, precapillary arterioles, and arteriovenous shunts of the skin causes the hands or feet to become white and numb and then bluish in color as blood flow remains blocked.

As the spasm eases and blood flow returns (approximately 10 to 15 minutes after the triggering stimulus has ended), rewarming occurs and the fingers or toes become red and painful. This cycle is initiated in response to stress or exposure to cold. Progressive phalangeal resorption may shorten the fingers, and compromised circulation resulting from abnormal thickening of the arterial intima may cause slowly healing ulcerations on the tips of the fingers or toes that may lead to gangrene.¹⁵⁸

Skin.: Other symptoms include pain, stiffness, and swelling of the fingers and joints. Skin thickening produces taut, shiny skin over the entire hand and forearm. As tightening progresses, contractures may develop. Flexion contractures are especially severe in people with dSSc (Fig. 10-23).

Figure 10-23 The progression of skin thickening in systemic sclerosis is greatest in the early period (first 5 years), especially in those with diffuse disease. (Modified from Clements PJ: Systemic sclerosis: natural history and management strategies, J Musculoskel Med 11[11]:43-50, 1994.)

Facial skin may also become tight and inelastic, and the face takes on a stretched and masklike appearance, with thin lips and a pinched nose. Peripheral nervous system involvement affects nerve terminals, reducing sensory fibers in SSc skin. Neuropeptides released by sensory nerve endings are reduced, resulting in vasoconstriction in the skin.

Neuromusculoskeletal System.: Most persons with dSSc have disuse atrophy of muscle because of limited joint motion secondary to skin, joint, or tendon involvement. A small percentage of people may have overlap syndromes and demonstrate marked weakness and inflammatory myopathy indistinguishable from polymyositis or dermatomyositis.

Some individuals develop myositis or erosive arthropathy that complicates the joint retraction induced by skin fibrosis. SSc also targets the peripheral nervous system with distal mononeuropathy of the median nerve as a frequent and early feature.⁵⁰ Neuropathy from carpal tunnel syndrome is also common.

Polyarthralgias affect both small and large joints and are especially frequent early in dSSc; polyarthritis is unusual. Tenosynovial involvement with inflammation and fibrosis of the tendon sheath or adjacent tissues is characterized by the presence of carpal tunnel syndrome and by coarse, leathery friction rubs palpated during motion over the extensor and flexor tendons of the fingers, distal forearms, knees, ankles, and other sites. These friction rubs are found almost exclusively in persons with dSSc, and their presence signifies a poorer overall clinical outcome.¹⁵⁸

Viscera.: GI motility dysfunction affects the esophagus and anorectal regions, causing frequent reflux, heartburn, dysphagia, and bloating after meals. Other effects include abdominal distention, diarrhea, constipation, and malodorous, floating stools. In advanced disease, cardiac and pulmonary fibrosis develops.

Cardiac involvement can be manifested as myocardial disease, pericardial disease, conduction system disease, or arrhythmias. Pulmonary involvement is characterized by impaired diffusing capacity for carbon monoxide. Kidney involvement and scleroderma renal crisis are now considered rare because of the introduction of angiotensin-converting enzyme (ACE) inhibitors.⁵⁰

Other.: Nearly 50% of adults with scleroderma report other symptoms, such as major depression, sexual dysfunction, trigeminal neuralgia, hypothyroidism, dental involvement, and corneal tears.

MEDICAL MANAGEMENT

DIAGNOSIS.

Early diagnosis and accurate staging of visceral involvement are fundamental for appropriate management and therapeutic approach to this disease. However, diagnosis can be delayed because there is no single laboratory test diagnostic for SSc. A thorough physical examination and history are the first steps to a definitive diagnosis.

Laboratory tests (skin biopsy, urinalysis, blood studies, including erythrocyte sedimentation rate [ESR], presence of rheumatoid factor [results found to be positive in 30% of SSc cases], presence of antinuclear antibodies) are used to determine the extent of involvement and rule out other disease processes. Distinctive serum autoantibodies are found in more than 90% of cases.⁹⁶ Other tests may include chest films and pulmonary function studies, GI series, and electroencephalogram (EEG).

TREATMENT.

Presently there is no cure for SSc. A global vision of SSc is necessary for this multisystem disease, and each treatment program is individualized to manage the specific disease process. Treatment ranges from merely symptomatic for a person with only limited skin involvement after 5 years to aggressive treatment for a person with early, diffuse skin involvement.

When organ involvement occurs, it most often develops early in the disease course, and in the acute phase it requires aggressive management. The program may include medications (e.g., immunosuppressants, penicillamine, antiinflammatory drugs), exercises, joint protection techniques, skin protection techniques, and stress management. See also the section on Raynaud’s Phenomenon in Chapter 12.

Penicillamine, a disease-modifying drug, is a penicillin-derivative immunomodulating agent that has been shown to improve the skin by interfering with cross linking of collagen and to prolong survival in people with early, rapidly progressive SSc. Oral tetracyclines have been found to be the most effective and have the fewest side effects, with minocycline or doxycycline being the antibiotic of choice. Brand-name drugs (Minocin, Vibramycin) are preferred because some generics are ineffective.¹⁵³

Treatment of the pulmonary complications (pulmonary hypertension, interstitial lung disease) remains difficult. Home blood pressure monitoring can screen for acute hypertension signaling a renal crisis; treatment with ACE inhibitors (see Chapter 12) may be lifesaving.

Research remains ongoing to investigate various treatment regimens for scleroderma, including the use of recombinant human relaxin to reduce skin thickening, improve mobility, and improve function in people with moderate to severe diffuse scleroderma.¹³⁷ The use of antibiotics such as minocycline without the use of any disease-modifying drugs or steroids has shown improvement in the disease, reduction of pain and severity of condition, and better quality of life.^25,84

PROGNOSIS.

The prognosis in SSc principally depends on early diagnosis; the intensity and rapidity of involvement of the lungs, heart, gut, and kidneys; and appropriate medical management. A model to predict mortality based on a combination of three factors (proteinuria, elevated ESR, low carbon monoxide diffusing capacity) has been reported to have an accuracy of more than 80% in predicting mortality. The absence of these three factors is associated with 93% survival.¹⁹

Spontaneous recovery is common in children, but approximately 30% of clients with SSc die within 5 years of onset. Persons with dSSc who have lived beyond the 5-year mark with no significant visceral involvement are unlikely to experience such organ involvement. Those in whom significant visceral disease developed early can expect a slowing in its progression or at least a stabilization of its course. This 5-year mark is also a time when skin softening begins and musculoskeletal aches and pains begin to ease.

Treatment with ACE inhibitors, started early, now prevents previously fatal complications (acute hypertension, renal failure). Aggressive treatment of early interstitial lung disease may further survival.⁵⁶ Localized scleroderma may reach an end point beyond which the disease does not progress.

10-13 SPECIAL IMPLICATIONS FOR THE THERAPIST

Systemic Sclerosis

Skin (Pruritus and Ulcers)

INTEGUMENTARY PRACTICE PATTERNS

7A:

Primary Prevention/Risk Reduction for Integumentary Disorders

7B:

Impaired Integumentary Integrity Associated with Superficial Skin Involvement

7C:

Impaired Integumentary Integrity Associated with Partial-Thickness Skin Involvement and Scar Formation

Itching can be a major problem in this condition, and excoriation from scratching can cause open wounds susceptible to infection. The therapist can offer some simple suggestions to soothe skin, ease the itching, and prevent skin damage (see Box 10-4).

Local management of digital tip ulcers may include an occlusive dressing to promote wound healing and protect against trauma and infection. Commercial occlusive dressings are particularly helpful with larger noninfected ulcers.

Infected ulcers are treated with a trial of oral antistaphylococcal antibiotics and may require surgical debridement of necrotic tissue. Local skin care requires avoidance of excessive bathing or using moisturizing creams containing glycerin. (See also the sections on Peripheral Vascular Disease: Special Implications for the Therapist, and Raynaud’s Phenomenon in Chapter 12).

Muscle

INTEGUMENTARY PRACTICE PATTERN

7E:

Impaired Integumentary Integrity Associated with Skin Involvement Extending into Fascia, Muscle, or Bone and Scar Formation

MUSCULOSKELETAL PRACTICE PATTERNS

4B:

Impaired Posture

4C:

Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Disorders

Myositis (muscle inflammation) is treated with corticosteroids and sometimes requires the addition of immunosuppressive drugs, whereas fibrotic myopathy (fibrotic tissue laid down within the muscle) is best managed with strengthening and ROM exercises. The efficacy of using soft tissue mobilization or similar techniques has not been investigated. Caution must be used when attempting such treatment, because the skin of these people is usually very sclerosed and sensitive to pressure. Aquatic therapy is an excellent choice for clients with this condition.

Joints and Tendons

INTEGUMENTARY PRACTICE PATTERN

7E:

Impaired Integumentary Integrity Associated with Skin Involvement Extending into Fascia, Muscle, or Bone and Scar Formation

MUSCULOSKELETAL PRACTICE PATTERN

4E:

Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation

Joint and tendon sheath involvement is common and may be treated successfully with nonsteroidal antiinflammatory drugs (NSAIDs). In early dSSc, tenosynovitis can be very painful, limiting joint movement. In addition to NSAIDs, early aggressive therapy is important in preventing or minimizing contractures.

For clients with scleroderma, regular exercise will assist with keeping the skin and joints flexible, maintaining better blood flow, and preventing contractures. Active and passive stretching exercises are necessary but difficult in the presence of extreme pain.

Analgesia is required to optimize participation in an exercise program. Protecting swollen and painful joints from stresses and strains is also an important factor. This may require teaching ways to carry out activities of daily living (ADLs) without causing strain on the joint or joints.

Lightweight splints may be necessary to provide joint protection. Dynamic splinting has not been found effective in preventing flexion contractures. Carpal tunnel syndrome, which often occurs before the diagnosis of scleroderma, usually responds well to conservative treatment without requiring surgery.

Exercise

CARDIOVASCULAR/PULMONARY PRACTICE PATTERNS

6B:

Impaired Aerobic Capacity/Endurance Associated with Deconditioning

6D:

Impaired Aerobic Capacity/Endurance Associated with Cardiovascular Pump Dysfunction or Failure

Practice patterns in this area will vary depending on the form of cardiovascular or pulmonary involvement manifested. When cardiopulmonary involvement occurs, intervention must take into consideration effects of this disease on the individual’s activity and lifestyle. The client’s primary diagnosis and primary intervention may be integument or orthopedics related, but functional limitations may be present secondary to systemic involvement (e.g., decreased aerobic capacity, endurance, and overall general physical condition secondary to cardiovascular and/or pulmonary involvement).

Psychologic Considerations

Persons with early dSSc with or without organ involvement are often anxious because their bodies are changing rapidly and in unexpected ways. They may not understand the grave nature of the disease.

Since persons with dSSc are at greatest risk for early visceral disease and early mortality, education about the disease is important, as is identifying where they are in the natural history of SSc. They should be encouraged to take their blood pressure at home at least three times per week, since this is the best method of screening for acute hypertension. The therapist may screen blood pressure as well.

Polymyositis and Dermatomyositis

Definition and Overview

Polymyositis and dermatomyositis are the two most common idiopathic inflammatory diseases of muscle (Box 10-14). Other types of inflammatory muscle disease have been distinguished, but no satisfactory classification of the idiopathic inflammatory myopathies exists; however, histologic analysis allows differentiation among the types of dermatomyositis.¹⁷ They are diffuse, inflammatory myopathies that produce symmetric weakness of striated muscle, primarily the proximal muscles of the shoulder and pelvic girdles, neck, and pharynx. These related illnesses belong to the family of rheumatic diseases. These diseases often progress slowly, with frequent exacerbations and remissions.

Box 10-14 TYPES OF INFLAMMATORY MYOPATHIES^*

• Primary idiopathic polymyositis

• Primary idiopathic dermatomyositis

• Dermatomyositis or polymyositis associated with malignancy (lung, breast, ovarian, gastric, colon)

• Juvenile polymyositis or dermatomyositis

• Polymyositis associated with other connective tissue diseases (overlap):

• Sjögren’s syndrome

• Mixed connective tissue disease

• Rheumatoid arthritis

• Systemic lupus erythematosus

• Scleroderma

^*Listed in descending order of frequency. Primary idiopathic polymyositis and dermatomyositis account for nearly three fourths of all cases.

Incidence

Polymyositis and dermatomyositis are not very common in the United States, affecting approximately 5 to 10 persons per 1 million; the incidence appears to be increasing. Myositis can affect people of any age, but mostly adults between 45 and 65 years and children between 5 and 15 years are affected. Twice as many women as men are affected with the exception of dermatomyositis associated with malignancy, which is most common in men over age 40 years.

Etiologic Factors

The cause of these conditions remains unknown, although there appears to be some autoimmune mechanism whereby the T cells inappropriately recognize muscle fiber antigens as foreign and attack muscle tissue. Autoantibodies are present in most cases. Polymyositis and dermatomyositis may be drug induced; possibly triggered by a virus; or associated with other disorders as listed in Box 10-14. The association of dermatomyositis with malignancy, particularly ovarian, gastric, and colonic malignancies, may suggest that the neoplasm may stimulate the dermatomyositis.¹⁷

Pathogenesis

If these conditions are caused by an autoimmune reaction, diffuse or focal muscle fiber degeneration is followed by regeneration of new muscle cells, producing remission. Muscle biopsy reveals focal or diffuse inflammatory infiltrates consisting primarily of lymphocytes and macrophages surrounding muscle fibers and small blood vessels. Muscle cells show evidence of degeneration and regeneration, and fiber atrophy is often most severe at the periphery of the muscle bundle. Extensive interstitial fibrosis and fatty replacement are common in longstanding cases.

Clinical Manifestations

Symmetric proximal muscle weakness is the dominant feature of these diseases, although it is variable in its onset, progression, and severity. In some people, symptoms appear suddenly, progress rapidly, and quickly result in a bedridden state, sometimes requiring ventilatory assistance and tube feeding.

More typically, malaise and weight loss develop insidiously over months or even years, with some people either unable to identify the onset of the disease or unaware of the gradual disability developing. Fatigue, rather than weakness, is a commonly reported symptom, but close questioning usually reveals functional losses indicating weakness as well. Pain is not a key feature of these diseases in the adult population, although aching muscles are not uncommon. Muscle wasting is observed in longstanding or severe cases.

Cardiac involvement is not uncommon and contributes significantly to mortality. Nearly half of all people with polymyositis or dermatomyositis have arrhythmias, congestive heart failure, conduction defects, ventricular hypertrophy, or pericarditis.

Pulmonary disease (progressive pulmonary fibrosis) can result from weakness of the respiratory muscles, intrinsic lung pathologic conditions, or aspiration. Swallowing difficulties, nasal regurgitation, and esophageal dysphagia and reflux are common, especially in severe cases.

Polymyositis.: Polymyositis begins acutely or insidiously with muscle weakness, tenderness, and discomfort. The proximal muscles of the shoulder and pelvic girdle are affected more often than the distal muscles, usually in a symmetric pattern, but asymmetry is common.

The legs are affected more often than the arms, and the anterior thigh is more frequently involved than the posterior thigh. Initially, the muscles may be slightly swollen, but as the disease progresses muscular atrophy and induration become more noticeable, reflecting the deposition of fibrous tissue. Some persons have a mild peripheral neuropathy with loss of deep tendon reflexes.

Early signs of muscle weakness may include impaired functional status, such as difficulty climbing stairs, getting up from a chair, reaching into an overhead cupboard, combing the hair, or lifting the head from a pillow; difficulty with balance; or a tendency to fall, often resulting in a fracture.

Other muscular effects may include decreased deep tendon reflexes, contractures, arthralgias, arthritis, an inability to move against resistance (e.g., pushing open a heavy door, opening a car door), proximal dysphagia (difficulty swallowing), and dysphonia (difficulty speaking).

Dermatomyositis.: When a rash is associated with polymyositis, it is referred to as dermatomyositis. A characteristic purplish rash appears on the eyelids (heliotrope erythema), accompanied by periorbital edema (puffy eyelids). The rash may progress to the anterior neck, upper chest and back, shoulders, and arms and may appear around the nail beds. Gottron’s papules (red or violet, smooth or scaly patches) may appear on the knuckles, elbows, knees, or medial malleoli (Fig. 10-24).

Figure 10-24 Gottron’s papules or Gottron’s sign. Typical lesions over bony prominences on the extensor surfaces of the hand. (From Bolognia JL, Jorizzo JL, Rapini RP: Dermatology, 2 volume set, St Louis, 2003, Mosby.)

Although the disease usually begins with erythema and swelling of the face and eyelids, cutaneous manifestations can develop concomitantly with muscle involvement or even afterward. The cutaneous lesions of dermatomyositis are nearly always present by the time proximal muscle weakness manifests itself. In some persons, muscle involvement is minimal, whereas in others it may progress to wasting and contractures associated with extreme disability.¹⁶³

MEDICAL MANAGEMENT

DIAGNOSIS AND TREATMENT.

Diagnosis is usually made based on the history and presenting symptoms; measures to prevent and treat general hypothermia are taken before managing the local frostbite injuries. Evidence of the role of thromboxanes and prostaglandins in cold injuries has resulted in more active approaches in the medical treatment of frostbite wounds, including the use of vasodilators, thrombolysis, and hyperbaric oxygen.¹⁰²

Triple-phase bone scans can be used to distinguish between tissue that is irreversibly destined for necrosis and tissue that is at risk for necrosis but potentially salvageable. These improvements in radiologic assessment have led to earlier surgical intervention to provide at-risk tissue with a new blood supply and preserve both function and length in an extremity.¹⁴⁶

In a localized cold injury, treatment consists of rewarming the injured part without rubbing or massaging the area to avoid further tissue damage, and supportive measures (e.g., analgesics for pain [200 mg of ibuprofen every 6 hours]¹²² and proper positioning to avoid weight bearing with gauze between the toes to prevent maceration). More severe and deeper injuries should not be thawed until medical treatment can be given in a hospital.

The management of blisters is still somewhat controversial, but current practice indicates that clear blisters (shallow injury) should be aspirated; hemorrhagic blisters (deep injury) should not be debrided to avoid desiccation and infection of underlying deep tissue²⁸ (see the section on Skin Lesions: Special Implications for the Therapist).

All frostbitten areas should be treated with topical aloe vera cream. Foam dressings may be applied to maintain a moist wound bed, absorb drainage, and provide protection. A bed cradle may be needed to keep the weight of bedcovers off the affected part or parts.

In the case of a developing compartment syndrome a fasciotomy may be performed to increase circulation by lowering edematous tissue pressure. If gangrene occurs, amputation may be necessary. Smoking causes vasoconstriction and slows healing; the client should be advised to quit smoking, at least during the recovery period.

PROGNOSIS.

The prognosis depends on the extent of localized cold injury and development of any complications, such as compartment syndrome, necrosis, or gangrene. Rapid triage and treatment of frostbite can lead to dramatic improvements in outcome and prognosis.¹²² Long-term effects may include increased sensitivity to cold, burning and tingling on reexposure to cold, and increased sweating of the affected area.

Future cold injuries may be prevented through the use of wind-proof, water-resistant, many-layered clothing; moisture-wicking socks; a head covering; mittens instead of gloves; and heat-generating devices (except for those with peripheral neuropathy) in pockets or batteryoperated socks.

10-15 SPECIAL IMPLICATIONS FOR THE THERAPIST

Cold Injuries

PREFERRED PRACTICE PATTERNS

4J:

Impaired Motor Function, Muscle Performance, Range of Motion, Gait, Locomotion, and Balance Associated with Amputation

7D:

Impaired Integumentary Integrity Associated with Full-Thickness Skin Involvement and Scar Formation

7E:

Impaired Integumentary Integrity Associated with Full-Thickness Skin Involvement Extending into Fascia, Muscle, or Bone and Scar Formation

Local cold injury subsequent to prolonged exposure may not be seen in a therapy practice until complications such as necrosis and gangrene result in amputation. Whirlpool with gentle agitation directed away from the affected area may be prescribed as part of the rewarming procedure. Water temperature is based on tissue temperature and should be determined in conjunction with the medical staff.

Use of cryotherapy as a modality among the general population can result in localized tissue damage requiring documentation (e.g., filing an accident report) and possible medical evaluation and treatment. Massage may cause further tissue damage and should not be carried out until local tissue has healed.

Burns

Definition and Overview

Injuries that result from direct contact with or exposure to any thermal, chemical, electrical, or radiation source are termed burns. Burn injuries occur when energy from a heat source is transferred to the tissues of the body. The depth of injury is a function of temperature or source of energy (e.g., radiation) and duration of exposure.

The severity of burn injury is assessed with respect to the risk of infection, mortality, and cosmetic or functional disability.¹¹⁷ Factors that influence injury severity include burn depth, burn size (percentage of total body surface area [TBSA]), burn location, age, general health, and mechanism of injury. Burn depth can be divided into categories based on the elements of the skin that are damaged (Fig. 10-26). Most burn wounds that require medical intervention are a combination of partial-and full-thickness burns.

Figure 10-26 Burn injury classification according to depth of injury. This information is important to review because it will help determine the practice pattern to use when making a physical therapy diagnosis. A partial-thickness burn involves loss of epidermis and/or a portion of the dermis. Because part of the dermis is intact and that is where the regenerating elements are, a partial-thickness wound has the ability to heal via epithelialization. A full-thickness burn involves total destruction of the epidermis and dermis and cannot heal independently without granulation and contraction, sometimes requiring a flap or skin graft procedure.³⁸

Burn size is determined by one of two techniques: the rule of nines (Fig. 10-27) and the Lund-Browder method (Figs. 10-28 and 10-29). The rule of nines is based on the division of the body into anatomic sections, each of which represents 9% or a multiple of 9% of the TBSA. This is an easy method to quickly assess the percentage of TBSA injured and is most commonly used in emergency departments where the initial evaluation takes place.

Figure 10-27 The rule of nines provides a quick method for estimating the extent of a burn injury.

Figure 10-28 A sample chart for recording the extent and depth of a burn injury using the Lund-Browder formula.

Figure 10-29 A, Pediatric scald burn. B, Corresponding Lund-Browder chart. (Courtesy Katherine S. Biggs, PT, Yale New Haven Hospital, New Haven, Conn.)

The Lund-Browder method modifies the percentages for body segments and provides a more accurate estimate of burn size according to age. For the most accurate estimate of burn size, the burn diagram should be confirmed following the initial wound debridement.⁹⁰

Incidence

In the United States, approximately 1.4 to 2 million burn injuries occur each year; 70,000 people are hospitalized with severe injuries; and 7500 are fatalities. Extensive autografts are required in over 1500 third-degree burns every year and 40,000 second-degree burns.

Burn injuries are the third leading cause of accidental death in all age groups. Males tend to be injured more frequently than females, except for the older population (older than 70 years).¹¹⁶ The incidence of burn injuries is expected to increase as an aging society characterized by a striving for independence becomes more apparent.⁹⁴

Etiologic Factors

Burn injuries are categorized according to their mechanism of injury: thermal, chemical, electrical, or radiation. Thermal burns are caused by exposure to or contact with sources such as flames, hot liquids, steam, semisolids (tar), or hot objects.

Chemical burns are caused by tissue contact with or ingestion, inhalation, or injection of strong acids, alkalis, or organic compounds. Chemical burns can result from contact with certain household cleaning agents and various chemicals used in industry, agriculture, and the military.

Electrical burns are caused by heat that is generated by the electrical energy as it passes through the body. Electrical burns can result from contact with exposed or faulty electrical wiring, high-voltage power lines, or lightning.

Radiation burns are the least common burn injury and are caused by exposure to a radioactive source. These types of injuries have been associated with the use of ionizing radiation in industry or with therapeutic radiation sources in medicine. A sunburn from prolonged exposure to UV rays is also considered a type of radiation burn.

Risk Factors

Data collected from the National Burn Information Exchange indicate that 75% of all burn injuries result from the actions of the injured person, occurring most often in the home. Children under 3 years and adults over 70 years are at the highest risk for burn injury.

Risk factors include inadequate adult supervision (in the case of children), psychomotor disorders (e.g., impaired judgment, impaired mobility, drug or alcohol use), rural location, mobile home residence, occupation, lack of smoke detectors, fireworks, and misuse of cigarettes.^27,94,95

New data demonstrate a change in the epidemiology of burns from previous studies. These data point out the relationship between epileptic seizures and domestic scald injuries. Scald injuries are now the major cause of burns in people with epilepsy and account for approximately 2% of all burn admissions.⁷²

Safety recommendations for prevention of burn injuries while showering have been made, including nonlever water handles, limited-temperature devices on water heaters, and curtains rather than cubicles for easy escape.¹⁵⁴

Pathogenesis

Cutaneous Burns.: The pathophysiologic changes that occur immediately following a cutaneous burn injury depend on the extent or size of the burn. For smaller burns, the body’s response to injury is localized to the injured area. With more extensive burns (25% or more of the TBSA), the response is systemic, potentially affecting all major systems of the body. The systems more obviously affected include the cardiovascular, renal, GI, immune, and respiratory systems.

Cardiovascular changes occur immediately following a burn injury as vasoactive substances (catecholamines, histamine, serotonin, leukotrienes, prostaglandins) are released from the injured tissue, causing an increase in capillary permeability.

Extensive burns result in generalized body edema in both burned and nonburned tissues and a decrease in circulating intravascular blood volume. Heart rate increases in response to catecholamine release and to the hypovolemia, but overall cardiac output falls. If the intravascular space is not replenished with IV fluids, hypovolemic (burn) shock and death may result.

Within 18 to 36 hours after the burn, capillary permeability decreases and continues to return to normal for several weeks following the injury. Cardiac output returns to normal and then increases approximately 24 hours after the injury to meet the hypermetabolic needs of the body. The body begins to reabsorb the edema fluid and excretes the excess fluid over the ensuing days and weeks. See also the section on Common Causes of Fluid and Electrolyte Imbalances in Chapter 5.

The renal and GI systems are affected as the body responds initially by shunting blood from the kidneys and intestines, leading to oliguria (decreased urine output) and intestinal dysfunction, respectively, in clients with burns of greater than 25% of TBSA. Immune system function is depressed, increasing the risk of infection and life-threatening sepsis. The respiratory system may respond with pulmonary artery hypertension and decreased lung compliance, even when there has been no inhalation injury.

Smoke Inhalation.: Smoke inhalation may result in injury secondary to inhalation of carbon monoxide, smoke poisoning from the inhalation of by-products of combustion, or direct thermal burns to the pulmonary airways. See the section on Noxious Gases, Fumes, and Smoke Inhalation in Chapter 15.

Electrical and Chemical Burns.: In electrical burns, heat is generated as the electricity travels through the body, resulting in internal tissue damage. However, entrance and exit wounds may be significant, distracting medical personnel from internal injuries.

Cutaneous burn injuries associated with electrical burns may be negligible, but soft tissue and muscle damage may be extensive, particularly in high-voltage electrical injuries. However, it is possible for electrical sources to ignite the person’s clothes, causing thermal burns as well.

The voltage, type of current (direct or alternating), contact site, and duration of contact are important factors in the amount and type of damage sustained. Alternating current is more dangerous than direct and is often associated with cardiopulmonary arrest, ventricular fibrillation, and tetanic muscle contractions.

Other significant injuries, such as long-bone or vertebral compression fractures, spinal cord injury, or traumatic brain injury, can occur if the victim falls on electrical contact. Chemical burns are associated with systemic toxicity from cutaneous absorption. See also Chapter 4.

Clinical Manifestations

Appearance, sensation, and course of injury of superficial, partial-thickness, and full-thickness burns are outlined in Fig. 10-26. Burn location influences injury severity in that burns of certain areas of the body are commonly associated with specific complications. For example, burns of the head, neck, and chest frequently have associated pulmonary complications.

Burns involving the face may have associated corneal abrasions. Burns of the hands (Fig. 10-30) and joints can result in permanent physical and vocational disability requiring extensive therapy and rehabilitation. Circumferential burns of extremities may produce a tourniquet-like effect and lead to total occlusion of circulation (Fig. 10-31).

Figure 10-30 Pediatric burn of the hand. This 2-year-old grabbed a hot iron, sustaining a partial-thickness burn to her hand. An intact blister remains on her middle finger. (Courtesy Harriett B. Loehne, PT, DPT, CWS, FCCWS, Archbold Center for Wound Management, Thomasville, GA, 2006. Used with permission.)

Figure 10-31 Circumferential burns of extremities may produce a tourniquet-like effect and lead to total occlusion of circulation. This 3-year-old child reached into the microwave oven and spilled macaroni in boiling water, resulting in a partial-thickness scald burn. (Courtesy Harriett B. Loehne, PT, DPT, CWS, FCCWS, Archbold Center for Wound Management, Thomasville, GA, 2006. Used with permission.)

Theoretically, with a full-thickness burn the nerve endings have been destroyed and no pain should be associated with this type of injury. However, most full-thickness burns occur with superficial and partial-thickness burns in which nerve endings are intact and exposed. Excised eschar (dead tissue) and donor sites expose nerve fibers as well. As peripheral nerves regenerate, painful sensation returns. Consequently, people with burn injuries often experience severe pain that is related to the size and depth of the burn.

The clinical course of the (major) burn client can be divided into three phases: the emergent and resuscitation phase, the acute phase, and the rehabilitation phase. The emergent period begins at the time of injury and concludes with the restoration of capillary permeability, usually 48 to 72 hours following injury.

The resuscitation period begins with initiation of fluid resuscitation measures and ends when capillary integrity returns to near-normal levels and the large fluid shifts have decreased. The acute phase of recovery begins when the person is hemodynamically stable, capillary permeability is restored, and diuresis has begun, usually 48 to 72 hours after the initial injury occurred. The acute phase continues until wound closure is achieved.

The rehabilitation phase represents the final phase of burn care, often overlaps the acute care phase, and lasts well beyond the period of hospitalization. This phase focuses on gaining independence through achievement of maximal functional recovery.

Infection is the most common and life-threatening complication of burn injuries. Burn wound infections can be classified on the basis of the causative organism, the depth of invasion, and the tissue response.

Individuals with extensive burns and in whom wound closure is difficult to achieve are at greatest risk for infection and other complications. Inhalation injury with major burns and added staphylococcal septicemia are often fatal.⁴⁷ The multiple organ system response that occurs following a burn injury may result in the multiple organ dysfunction syndrome and death (see Chapter 5).

Hypertrophic scarring is a second complication that although not life-threatening is associated with considerable morbidity and potential lifelong disfigurement. Children and African Americans are at greatest risk for hypertrophic scarring, presumably because of the abundance of collagen in these groups. Aging Caucasian adults with wrinkled, loose skin have little to no hypertrophic scarring because of the absence of collagen.

MEDICAL MANAGEMENT

TREATMENT.

The therapist may be involved in wound care for minor burns consisting of cleansing; removal of any damaging agents (e.g., chemicals, tar); debridement of loose, nonviable tissue; and application of topical antimicrobial creams or ointment and a sterile dressing. Blister management usually includes debridement of the blister (see the section on Skin Lesions: Special Implications for the Therapist 10-1). Although the blister fluid is theoretically sterile, most blisters break, and the fluid is an ideal medium for bacteria.¹³⁰

Instructions for home care include observation for clinical manifestations of infection and active ROM exercises to maintain normal joint function, decrease edema formation, and decrease possible scar formation.

Treatment of major burns includes lifesaving measures (ABCs: airway, breathing, circulation) immediately after the injury followed by restorative care (e.g., infection control, wound care, skin grafts, pain management) during the acute phase until wound closure is achieved. Therapists are closely involved early in the acute phase of recovery to maximize functional recovery and cosmetic outcome.

Therapeutic interventions include wound management—irrigation, debridement, advanced wound dressings, positioning and immobilization following skin grafting to prevent unwanted movement and shearing of grafts, scar and contracture prevention and management, exercise, ambulation, and ADLs. Elasticized garments help reduce scar hypertrophy and may be worn for months to 2 years after hospitalization.

Bioengineered temporary biologic dressings may be used to minimize fluid and protein loss from the burn surface, prevent infection, and reduce pain. Types of temporary grafts include allografts (homografts), which are usually cadaver skin; xenografts (heterografts), which are typically pigskin; and biosynthetic grafts, which are a combination of collagen and synthetics. To treat a full-thickness burn, an autograft (the person’s own skin) may be required.

The transplanted skin graft will be used intact over areas where appearance or joint movement is important, but the graft may be meshed (fenestrated) to cover up to three times its original size. Several new permanent skin substitutes are being utilized to aid in replacing dermal thickness and to assist in coverage of large surface area injuries.¹¹⁵ Cultured skin is usually used in conjunction with allograft dermis. See the section on Skin Transplantation in Chapter 21.

PROGNOSIS.

Burn care has improved in recent decades, resulting in a lower mortality rate for victims of burn injuries. Current techniques of burn wound management, such as effective topical antimicrobials and early burn wound excision, have significantly reduced the overall occurrence of invasive burn wound infections.¹¹⁷

The client’s age affects the severity and outcome of the burn. Mortality rates are higher for children less than 4 years of age and for clients over 65 years, although survival rates after burns have improved significantly for children. At present most children, even children with large burns, should survive.¹³⁸ Survival rate for older clients is 70%, with at least 60% of those individuals becoming fully functional 6 months after hospital discharge.⁹⁴

Factors such as obesity, alcoholism, and cardiac disorders affecting general health, especially disorders that impair peripheral circulation, such as peripheral vascular disease, increase the complication and mortality rates for adults with burns.

Delay in amputation results in prolonged hospital stay, delayed rehabilitation, and a higher mortality rate. Early amputation is associated with a 14% mortality rate compared with a 50% mortality rate for cases of delayed amputation. Earlier identification of nonsalvageable limbs may decrease infectious complications and improve chances of survival.¹⁶⁶

10-16 SPECIAL IMPLICATIONS FOR THE THERAPIST

Burns

PREFERRED PRACTICE PATTERNS

6C:

Impaired Ventilation, Respiration/Gas Exchange, and Aerobic Capacity/Endurance Associated with Airway Clearance Dysfunction

6E:

Impaired Ventilation and Respiration/Gas Exchange Associated with Ventilatory Pump Dysfunction or Failure

7B:

Impaired Integumentary Integrity Associated with Superficial Skin Involvement

7C:

Impaired Integumentary Integrity Associated with Partial-Thickness Skin Involvement and Scar Formation

7D:

Impaired Integumentary Integrity Associated with Full-Thickness Skin Involvement and Scar Formation

7E:

Impaired Integumentary Integrity Associated with Skin Involvement Extending into Fascia, Muscle, or Bone and Scar Formation

In light of statistics showing that the population over 70 years old is at highest risk of burn injury, prevention of burn accidents, especially in this population, is an important part of client education. Additional resources for the therapist are available.^{23,108,125,161}

Reviewing simple cooking precautions may be helpful; for example, do not leave burners in use unattended, do not use high heat, do not wear clothing with loose sleeves or belts (especially bathrobes), use front burners whenever possible, and avoid leaning over front burners when using back burners.

Unless employed in an emergency department, most therapists do not begin to treat the burn client until the acute phase (as soon as the person is physiologically stable), continuing intervention through much of the rehabilitation phase. However, initiating bedside intervention before the person is medically stable is ideal in reducing morbidity and functional loss.

The therapist will direct treatment intervention to encourage deep breathing and facilitate lung expansion; promote wound healing; reduce dependent edema formation and promote venous return; prevent or minimize deformities and hypertrophic scarring; increase ROM, strength, and function; increase independence in daily activities and self-care; and encourage emotional and psychologic well-being. Specific compression, lymphatic movement, debridement, and wound care procedures are beyond the scope of this book; the reader is referred to other texts for more detailed information.^{23,63,93,125,147}

Throughout the acute and rehabilitation phases of burn care, the therapist must remain alert to the development of medical complications, such as ileus, gastric ulcers, respiratory distress, infection, and impaired circulation. Monitor vital signs (e.g., heart rate, blood pressure, oxygen saturation levels) to ensure the person can tolerate therapy. Notify the nursing staff of any new or unusual findings observed during assessment and intervention (Table 10-6).

Table 10-6

Assessing Medical Complications in the Burn-Injured Adult

Laboratory values will change with a burn, especially a full-thickness burn. It is important for the therapist to review the values prior to treatment interventions to be aware of response to treatment and possible mental status changes. Lab values listed in Table 10-7 should be reviewed.

Table 10-7

Laboratory Values Associated with Burns

^*Hyponatremia (sodium imbalance) is seen initially due to extracellular changes following changes in cellular permeability. Fluid replacement is initiated to manage both fluid and electrolyte imbalances; the use of a number of solutions can effectively manage this deficit. Hypernatremia can occur as a result of dehydration, which explains why some patients can have hyponatremia and then develop hypernatremia.

Data from Ramos CG: Management of fluid and electrolyte disturbances in the burn patient, Annals of Burns and Fire Disasters, 8(4), 2000 [electronic version]. Available on-line at http://www.medbc.com/annals/review/vol_13/num_4/text/vol13n4p201.htm. Accessed October 25, 2006. From Demling RH: The Burn Nutrition Module: what are standard clinical monitors used to assess adequate nutrition? Available on-line at http://www.burnsurgery.com/Modules/burnnutrition/sec8.htm. Accessed October 25, 2006.

Clients with burns with acute renal failure and abnormal sodium, potassium, chloride, and magnesium values are candidates for hemodialysis. Abnormal BUN (blood urea nitrogen) can be a reflection of decreased renal function or fluid intake. A client may respond to physical therapy treatment with a decreased mental status. Because of the increased metabolism and catabolic state, clients will experience increased weakness.

Wounds will not heal optimally without addressing protein status, reflected in prealbumin, which will be affected by the catabolic state. Glucose must be monitored due to the same metabolic situation.

Regular inspection of the wound must be made; any change in wound appearance must be reported. The amount of body surface area exposed during wound care must be minimized to prevent hypothermia, because heat is lost in open wounds and after hydrotherapy by evaporation. Hydrotherapy treatment must be limited to 30 minutes or less with water temperature in the 98° to 102° F (36.7° to 38.9° C) range if whirlpool is used. External heat shields or radiant heat lamps can provide a source of external heat.

Clients excluded from hydrotherapy are generally those who are hemodynamically unstable and those with new grafts. In recent years, hydrotherapy has been challenged, and alternative methods are being advocated (e.g., shower versus tub).⁸⁷ Pulsed lavage with suction (PLWS) (Fig. 10-32) is an ideal intervention for irrigation and debridement, allowing treatment in the Burn Unit of appropriate areas without disturbing new grafts.⁸⁷

Figure 10-32 Personal protective equipment (PPE) worn during treatment with pulsed lavage with suction (PLWS). (Courtesy Harriett B. Loehne, PT, DPT, CWS, FCCWS, Archbold Center for Wound Management, Thomasville, GA, 2006. Used with permission.)

People with burns are at high risk of infection because of the significant loss of skin barrier and impaired immune response. Infection control techniques must be practiced carefully at all times (see Appendix A). Skin donor sites require the same care and precautions as other partial-thickness wounds in order to promote healing and prevent infection.

Arrange any therapy likely to elicit a painful response to coincide with medications (allow 30 minutes for oral, 10 minutes for intramuscular [IM], 3 to 5 minutes for IV administration). Combining relaxation techniques, music therapy, distraction, and other techniques for pain modulation may be helpful. Burned areas must be maintained in positions of physiologic function within the limits imposed by associated injuries, grafting, and other therapeutic devices (see Table 10-8 for positioning recommendations).

Table 10-8

Therapeutic Positioning for the Burn-Injured Client

Modified from Carrougher GJ: Nursing care of clients with burns. In Black JM, Matassarin-Jacobs E, eds: Medical-surgical nursing, ed 5, Philadelphia, 1997, Saunders, p 2260.

Burned areas are prone to develop contractures requiring close assessment of ROM and muscle strength. Encourage active ROM exercises at least every 2 hours while the person is awake unless this is contraindicated by a recent grafting procedure.

Prolonged stretching is sometimes combined with splinting or orthoses to maintain motion. Splinting is sometimes controversial due to the lack of evidence validation, although most clinicians employ splints successfully to prevent contractures.¹²⁶

Provide honest, positive reinforcement throughout intervention, being aware that each individual will progress through stages of denial, grief, and acceptance of injury and recovery. During the rehabilitation phase, chronic pain protocols may be helpful (see Box 3-15).

MISCELLANEOUS INTEGUMENTARY DISORDERS

Integumentary Ulcers

Integumentary ulcers can be caused by a variety of underlying disorders, including diabetes, arterial insufficiency, radiation damage, SSc, vasculitis, and prolonged pressure. In keeping with the focus of this text of recognizing the underlying pathology for various conditions, integumentary ulcers are discussed in individual sections according to the pathogenesis (e.g., diabetic ulcers, see the section on Diabetes Mellitus in Chapter 11; arterial insufficiency ulcers, see the section on Peripheral Vascular Disease in Chapter 12).

Pressure Ulcers

Definition and Overview

A pressure ulcer (formerly called bed sore, decubitus ulcer) is a lesion caused by unrelieved pressure resulting in damage to underlying tissue. Pressure ulcers usually occur over bony prominences, such as the heels, sacrum, ischial tuberosities, greater trochanters, elbows, and scapula, and are staged to classify the degree of tissue damage observed (Fig. 10-33; Box 10-15).

Box 10-15 STAGES OF PRESSURE ULCERS

Stage I^*

An observable pressure-related alteration of intact skin that, when compared to an adjacent or opposite area on the body, shows changes in one or more of the following:

• Skin temperature (warmth or coolness)

• Tissue consistency (firm or boggy feel)

• Sensation (pain, itching)

The ulcer appears as a defined area of persistent redness in lightly pigmented skin, whereas in darker skin tones, the ulcer may appear with persistent red, blue, grey, or purple hues.

Note: Reactive hyperemia normally can be expected to be present for one half to three fourths as long as when the pressure-occluded blood flow to the area is restored; it should not be confused with a stage I pressure ulcer.

Stage II

Partial-thickness skin loss involving epidermis and/or dermis. The ulcer is superficial and presents clinically as an abrasion, blister, or shallow crater.

Stage III

Full-thickness skin loss involving damage or necrosis of subcutaneous tissue that may extend down to, but not through, underlying fascia. The ulcer presents clinically as a deep crater with or without undermining of adjacent tissue.

Stage IV

Full-thickness skin loss with extensive destruction, tissue necrosis, or damage to muscle, bone, or supporting structures (e.g., tendon, joint capsule).

Note: Undermining and sinus tracts may also be associated with stage IV pressure ulcers.

Unstageable

The depth of a wound with eschar or more than 50% necrotic tissue cannot be determined until the necrotic tissue is debrided; it cannot be staged. Wounds that qualify as unstageable have eschar, are covered with necrotic tissue such that the base cannot be determined, or are filled with granulation tissue. These wounds are at least stage III or stage IV.

Proposed New Stage: Deep Tissue Injury

The National Pressure Ulcer Advisory Panel (NPUAP) has added a new stage (deep tissue injury, or DTI) for pressure ulcers with the skin intact, a purple color, and the appearance of a deep bruise (Fig. 10-34). It is a pressure-related injury to the subcutaneous tissues under intact skin. These lesions may herald the subsequent development of a stage III or IV pressure ulcer even with optimal treatment (NPUAP, 2006).

Figure 10-34 Deep tissue injury (DTI). A DTI pressure ulcer presents suddenly as a discolored/bruised-appearing area that quickly progresses to a deep wound regardless of any intervention. It is an unavoidable ulcer and usually occurs when other organs are shutting down. Because the skin is the largest organ in the body, necrosis of the skin will occur when circulation is significantly impaired. A, The DTI was discovered when an unstable patient in the intensive care unit was finally able to be turned over after 2 days. He had kidney failure, was on a ventilator, and had tenuous blood pressure. The wound presented as a discolored area with intact skin (formerly required to be staged as a stage I pressure ulcer). B, One week later it was solid eschar, which required sharp debridement due to purulent drainage at the margins and was necrosed to bone. The patient expired several days later. (Courtesy Harriett B. Loehne, PT, DPT, CWS, FCCWS, Archbold Center for Wound Management, Thomasville, GA, 2006. Used with permission.)

Data from U.S. Department of Health and Human Services: Pressure ulcers in adults: prediction and prevention. Clinical practice guideline no. 3. AHCPR publication no. 92-0047, Rockville, MD, 1992, The Department.

National Pressure Ulcer Advisory Panel: 2006. Updated staging system. Available on-line at www.npuap.org. Accessed April 19, 2008.

Note: Wounds cannot be backstaged (see text for explanation).

^*Revised 1998 stage I pressure ulcer definition; the NPUAP has not changed the definitions of stages II to IV pressure ulcers.

Figure 10-33 Staging pressure ulcers is based on the depth and type of tissue damage. This system was developed by the National Pressure Ulcer Advisory Panel (NPUAP).

In 1975 a landmark paper was published describing a method of classifying pressure ulcers, defined by the anatomic depth of soft tissue damage^137a (see Fig. 10-33). Since that time the original staging system has been modified and developed into the current staging system adopted by the Agency for Health Care Policy and Research (AHCPR) Pressure Ulcer Guideline Panels and published in both sets of Pressure Ulcer Clinical Practice Guidelines.^9,65 The revised stage I definition adopted by the National Pressure Ulcer Advisory Panel (NPUAP) in 1998 that is more inclusive of the range of skin pigmentation is included in Box 10-15.

Wounds cannot be backstaged. Once a pressure ulcer is designated as stage II, III, or IV, it will always remain classified the same for documentation. As the lesion fills with granulation tissue and closes with epithelial tissue, grafts, or flaps, it should be documented as healing stage II, III, or IV (still using the original deepest level noted). Some agencies require backstaging for reimbursement purposes; hopefully this will be changed in the future so that no backstaging will exist in any form.

It should be noted that this staging classification is only for pressure ulcers. Other types of ulcers, such as vascular (arterial, venous) are designated partial or full thickness. Neuropathic ulcers (see Table 12-20) are staged using Wagner’s classifications (Table 10-9). The term neuropathic ulcer is used interchangeably with diabetic ulcer, but a diabetic ulcer is really a neuropathic ulcer in someone with diabetes. Neuropathic ulcers can occur in anyone with loss of sensation (e.g., alcoholic neuropathy, peripheral neuropathy).

Table 10-9

Wagner’s Ulcer Grade Classification

Grade	Characteristics
0	Preulcerative lesions; healed ulcers; presence of bony deformity
1	Superficial ulcer without subcutaneous tissue involvement
2	Penetration through the subcutaneous tissue; may expose bone, tendon, ligament, or joint capsule
3	Osteitis, abscess, or osteomyelitis
4	Gangrene of digit
5	Gangrene of foot requiring disarticulation

This classification scheme for ulceration is used for neuropathic ulcers and does not represent pressure ulcers. It is included here for comparison with the stages of pressure ulcers (see Box 10-15).

From Wagner REW: The dysvascular foot: a system for diagnosis and treatment, Foot Ankle:64-122, 1981.

Incidence

Consistent data concerning incidence of pressure ulcers in the United States are difficult to find. Studies differ in a number of significant ways, and methodologies vary considerably.^10,11 Given these limitations it is estimated that 1.8 million³⁴ people develop pressure ulcers each year, including 500,000 people in nursing homes and another 400,000 people with diabetic foot ulcers.

Pressure ulcers are viewed as high-volume, high-risk problems in most health care settings. In long-term care facilities, regulatory agencies have designated the development of pressure ulcers as an indicator of quality of care provided to clients.⁴⁴

Healthy People 2010 has set as an objective to reduce the proportion of nursing home residents with current diagnosis of pressure ulcers from 16 per 1000 residents to no more than 9 per 1000 residents.

The target-setting method used by Healthy People 2010 in conjunction with the NPUAP is based on a 50% reduction in prevalence and improvement over the baseline. Incidence refers to the rate at which new cases occur in a population over a given period of time, whereas prevalence refers to the number of both new and old cases at any one time in the population.

In 2005 the Centers for Medicare and Medicaid Services (CMS) issued new guidelines for nursing homes, giving surveyors specific criteria for inspections. Included is guidance for F Tag 314 for the prevention, assessment, and treatment of pressure ulcers in nursing homes.²⁶ It is anticipated that hospitals will be held to the same standards in the near future.

Etiologic and Risk Factors

Pressure ulcers are caused by unrelieved pressure that results in damaged skin, muscle, and underlying tissue, usually over bony prominences. The primary causative factors for the development of pressure ulcers are (1) interface pressure (externally); (2) friction (rubbing of the skin against another surface); (3) shearing forces (two layers sliding against each other in opposite directions causing damage to the underlying tissues); (4) maceration (softening caused by excessive moisture); (5) decreased skin resilience (e.g., dehydration); (6) malnutrition; and (7) decreased circulation.

Pressure contributes to other types of ulcers (e.g., arterial, venous, neuropathic), and likewise, the underlying cause of the other types of ulcers can contribute to the development of pressure ulcers (see Table 12-20). However, pressure ulcers are a separate entity from these other types of ulcers. A systemic risk assessment evaluating both sensation and physiologic risk of pressure ulcers can be made using a validated risk assessment tool, such as the Braden Scale (Table 10-10) or the Norton Scale (Table 10-11).

Table 10-10

The Braden Scale for Predicting Ulcer Risk

Key: A score of 15 to 16 (15 to 18 if >75 yr) indicates minimum risk; 13 to 14, moderate risk; ≤12, high risk. IV, Intravenous; NPO, nothing by mouth; TPN, total parenteral nutrition.

Copyright Barbara Braden and Nancy Bergstrom, 1988. Used with permission of Braden B, Frantz R: Selecting a tool to measure skin integrity. In Stromberg M, ed: Instruments for clinical nursing research, ed 2, Norwalk, CT, 1997, Appleton and Lange.

Table 10-11

Norton Scale

Key: The Norton Scale is a summated rating scale made up of five subscales scored from 1-4 (1 for low level of functioning and 4 for highest level functioning), for total scores that range from 5-20. The subscales measure functional capabilities of the person that contribute to their risk of developing pressure ulcers. A lower Norton Scale score indicates lower level of functioning and, therefore, higher level of risk for pressure ulcer development. A score of 5 to 14 rates the client “at risk.”

From Norton D, McLaren R, Exton-Smith AN: An investigation of geriatric nursing problems in the hospital, London, 1962, National Corporation for the Care of Old People (now the Centre for Policy on Ageing).

Intrinsic factors most commonly associated with pressure ulcer development include decreased sensation, impaired mobility or activity levels, incontinence, diaphoresis, impaired nutritional status, and altered levels of consciousness. Extrinsic factors include pressure, shear, friction, and moisture.

Bed-and chair-bound clients and those with impaired ability to reposition themselves should be assessed for additional factors that increase the risk of developing pressure ulcers. These factors include decreased mobility or immobility; hip or femoral fractures; contractures; increased muscle tone; loss of sensation; incontinence; obesity; nutritional factors; chronic disease accompanied by anemia, edema, renal failure, or sepsis; and altered level of consciousness.

Nutritional factors may include malnutrition or inadequate nutrition leading to weight loss and subsequent reduction of subcutaneous tissue and muscle bulk. The Agency for Health Care Policy and Research guidelines⁹ for clinically significant malnutrition impairing wound healing include serum albumin less than 3.5 mg/dl and total lymphocyte count less than 1800/mm³ (see Tables 40-5 and 10-11).

Prealbumin, which determines protein over the previous 48 hours, rather than over the previous 3 weeks as with albumin, is a better indicator of the current nutritional status of the client. Prealbumin should be more than 20 for optimal wound healing. It is considered the gold standard for monitoring nutritional progress, allowing for documentation and appropriate interventions.³¹

Pathogenesis

Pressure is the external factor causing ischemia and tissue necrosis. Continuous pressure on soft tissues between bony prominences and hard or unyielding surfaces compresses capillaries and occludes blood flow. Normal capillary blood pressure at the arterial end of the vascular bed averages 32 mm Hg.

When tissues are externally compressed, that pressure may be exceeded, reducing blood supply to, and lymphatic drainage of, the affected area.^64,65 Shearing (when the skin layers move in opposite directions) is the intrinsic factor that contributes to ripping or tearing of blood vessels, further damaging the integument.

If the pressure is relieved, a brief period of rebound capillary dilation (called reactive hyperemia) occurs and no tissue damage develops. If the pressure is not relieved, the endothelial cells lining the capillaries become disrupted by platelet aggregation, forming microthrombi that occlude blood flow and cause anoxic necrosis of surrounding tissues. Necrotic tissue predisposes to bacterial invasion and subsequent infection, preventing healthy granulation. Muscle and tendon tissue can tolerate less pressure loading than skin before incurring ischemic damage (Fig. 10-35).⁹¹

Figure 10-35 Evolving stages of a stage IV pressure ulcer. This diagram represents a trochanter partially surrounded by tendon, with subcutaneous, dermis, and epidermis layers (vertical lines left to right). As pressure occurs from the outside (arrow), the tendon becomes ischemic first, and then the subcutaneous layer is affected because it is less vascular than the dermis. The last tissue to become ischemic is the epidermis. The observer would initially identify the epidermal tissue change as a stage I pressure ulcer. Initially, the ischemic inner tissue layers would not be known. Only as the necrosis moves superficially will the full impact of tissue damage become observable, identifying the area as a stage IV pressure ulcer with extensive damage to the bone. (Courtesy Karen Kendall, PT, CWS, Medical Center for Continuing Education, Gulf Breeze, FL, 2000.)

In the case of neuropathic ulcers associated with diabetes, the primary pathogenesis is the absence of protective sensation combined with high pressure. The absence of protective sensation indicates a high risk for pressure ulcers on the feet; diabetic ulcers are typically present on the soles of the feet (see the section on Diabetes Mellitus: Ulceration in Chapter 11).

Clinical Manifestations

Pressure ulcers usually occur over bony prominences and often in a circular pattern shaped like an inverted volcano with the greatest tissue ischemia at the apex next to the bone, or they may assume the shape of objects causing the pressure, such as tubing or clamps. Irregular patterns indicate additional shearing forces or other contributing factors.

Sacral ulcers are often large, undermined, and deep to the bone since the tissue mass over the sacrum is thin and erodes easily to the deep tissues. Pressure ulcers are manifested at the surface as the deeper tissues die, so that a stage I ulcer can become a stage III or IV quickly without further injury.

The wounds (Fig. 10-36) can be described, measured, and categorized with respect to surface area, exudates, and type of wound tissue. Therapists may want to utilize the PUSH Tool to assess and document pressure ulcers. This tool is available from the National Pressore Ulcer Advisory Panel (NPUAP): www.npuap.org. When present, infection can be localized and self-limiting or can progress to sepsis. Proteolytic enzymes from bacteria and macrophages dissolve necrotic tissues and cause a foul-smelling discharge that appears like, but is not, pus.

Figure 10-36 Unstageable (due to eschar) trochanteric pressure ulcer. (Courtesy Harriett B. Loehne, PT, DPT, CWS, FCCWS, Archbold Center for Wound Management, Thomasville, GA, 2006. Used with permission.)

Necrosis associated with pressure ulcers is not painful, but the surrounding tissue is often painful in individuals who do not have loss of sensation from spinal cord trauma or neuropathy. Trauma to the tissues produces an acute inflammatory response with hyperemia, fever, and increased white blood cell count.

Many individuals never initiate a significant acute inflammatory response because of the heavy bioburden from large amounts of necrotic tissue, but develop an unresolved chronic inflammation. Individuals who are immunosuppressed or who have diabetes mellitus are often unable to mount a sufficient inflammatory response to start the healing cascade and thus are at greater risk for infection.

MEDICAL MANAGEMENT

DIAGNOSIS.

Prevention is the key to this condition (Box 10-16), starting with assessment of people at high risk for the development of pressure ulcers. In fact, risk prediction should be an ongoing assessment carried out by all health care professionals. In addition to the Braden Scale (see Table 10-10) or Norton Scale (see Table 10-11), laboratory data on hemoglobin, hematocrit, prealbumin, total protein, and lymphocytes should be assessed by all health care professionals involved.

Box 10-16 GUIDELINES FOR PREVENTION OF PRESSURE ULCERS IN ADULTS

• All clients at risk should have a systematic skin inspection at least once each day, paying particular attention to the bony prominences. Results of skin inspection should be documented (see Box 10-5).

• Clean skin at the time of soiling and at routine intervals. Individualize the frequency of skin cleaning according to need and client preference. Avoid hot water, and use a mild cleaning agent that minimizes irritation and dryness of the skin. During the cleaning or wound care process, minimize the force and friction applied to the skin. Ideal is the use of disposable perineal cloths, impregnated with dimethicone.

• Minimize environmental factors leading to skin drying, such as low humidity (<40%) and exposure to cold. Treat dry skin with moisturizers.

• Do NOT perform massage over reddened areas. Perform indirect soft tissue mobilization techniques or massaging the tissue around and toward the area with caution.

• Minimize skin injury caused by friction and shear forces through proper positioning, transferring, and turning techniques. Reduce friction injuries by the use of moisturizers, transparent film dressings, skin sealants, and protective padding.

• Maintain current activity level, mobility, and range of motion. Evaluate the potential for improving the person’s mobility and activity status, and institute rehabilitation efforts.

• Monitor and document interventions and outcomes.

• If abnormal (<20 g/dl), order prealbumins one to two times weekly and have dietitian follow to optimize nutrition.

• Reposition any person in bed who is assessed to be at risk of developing pressure ulcers at least every 2 hours if consistent with overall treatment goals.

• For persons in bed, use positioning devices such as pillows or foam wedges to keep bony prominences (e.g., knees, ankles) from direct contact with one another.

• Provide persons in bed who are completely immobile with devices that completely relieve pressure on the heels (i.e., suspend the heels).

• Do not use doughnut-type devices.

• When side-lying position is used in bed, avoid positioning directly on the greater trochanter.

• Maintain the head of the bed at the lowest degree of elevation (30-to 35-degree lateral incline; see Fig. 10-38) consistent with medical conditions and other restrictions, except at mealtimes. Limit the amount of time the head of the bed is elevated.

• Use lifting devices, such as a trapeze, hydraulic lift, slide board, or linen, to move (rather than drag) persons in bed who cannot assist during transfers and position changes.

• Place any person assessed to be at risk of developing pressure ulcers, when lying in bed, on a pressure-redistribution surface, such as foam, air loss, gel, or water mattress.

• Avoid uninterrupted sitting in any chair or wheelchair for any person at risk of developing a pressure ulcer. Reposition the person, shifting the points under pressure at least every hour, or put him or her back to bed if consistent with overall management goals. Persons who are able should be taught to shift weight every 15 minutes.

• For chair-bound persons, use a pressure-redistribution device. Do not use doughnut-type devices.

Modified from Panel on the Prediction and Prevention of Pressure Ulcers in Adults: Pressure ulcers in adults: prediction and prevention. Clinical practice guidelines. CPR publication no. 92-0050, Rockville, MD, 1992, Agency for Health Care Policy and Research, Public Health Service.

The diagnosis is reached by looking at the location of the wound and the type of tissue response. The pressure ulcer is then staged (see Box 10-15 and Table 10-10). If there is evidence of infection, the wound is cleaned with isotonic saline and debrided if necrosis is present, and then viable tissue is cultured (not a swab specimen of the exudates or necrotic tissue).

The definition of infection is invasion into viable tissue. Cultures of the organisms that have invaded the tissue causing the infection must be determined following these procedures. Clinical practice of wound cultures must be careful to avoid culturing wound exudate contaminants, of which there are usually a minimum of three per wound.⁷⁶

Sensitivity testing to identify infecting organisms and to help determine appropriate topical or systemic antibiotics may be needed. The AHCPR (No. 15) recommends blood cultures for ulcer-related sepsis to determine appropriate systemic antibiotics.

TREATMENT.

Prevention and removing the causative factor are the first step in the treatment intervention for pressure ulcers. Preventing shear and friction forces requires education of the client and primary caretakers. The pressure ulcer is cleansed thoroughly. Healing will occur optimally when the ulcer is kept moist.

Topical antibiotics (e.g., Polysporin, Neosporin, bacitracin, Bactroban, MetroGel) can be effective on local infections without systemic involvement to control bacterial concentration, being mindful of allergic reactions, especially to neomycin and bacitracin. Antiseptics are not recommended because these are cytotoxic.

Some physicians continue to advocate the initial use of wet-to-dry dressing for debridement (application of open wet dressing, allowing it to dry on the ulcer, and mechanically debriding exudate by removal of the dressing). Because there is a risk of removing viable tissue, damaging new granulation tissue, as well as bleeding with this procedure, it is not acceptable for debridement if any viable tissue is evident and should be used only rarely. Wet-dry is not permissible as a dressing change order—only for debridement.^8,75

The use of antiseptics such as hydrogen peroxide or povidone iodine (cadexomer iodine is an excellent antimicrobial dressing choice) is not recommended because these are cytotoxic and can be damaging to granulation tissue. Hyperbaric oxygen therapy (HOT) has not been approved for pressure ulcers except when osteomyelitis is present that has failed systemic antibiotic treatment or there are complications from a flap or graft.

Successful healing requires continued adequate redistribution of pressure (e.g., turning, positioning, support surfaces) and absence of infection. The presence of necrotic tissue in a wound may provide an optimal environment for bacteria to grow, hence the importance of removing necrotic material from a wound as rapidly as possible.

Therapeutic intervention may include hydrotherapy (e.g., PLWS; see Fig. 10-32), electrical stimulation, ultrasound, debridement (autolytic, enzymatic, mechanical, sharp), or any combination of these. An appropriate wound dressing is then applied to provide an optimal wound environment.

Large deep pressure ulcers may require sharp or surgical debridement of necrotic tissue and opening of deep pockets for drainage. A slower method of debridement is the use of proteolytic enzymes. A variety of skin-grafting techniques may be used if the wound requires surgical closure.

In stage III ulcers, undamaged tissue near the wound is rotated to cover the ulcer. In stage IV ulcers, musculoskeletal flaps (a single unit of skin with its underlying muscle and vasculature), as well as a variety of other skin-grafting techniques, may be used effectively to close the wound.

Bioactive human dermal tissue capable of interacting with the wound bed is now available commercially for use in pressure and neuropathic ulcer wound management.

These skin substitutes derived from living human tissue (human fibroblasts) represent an important advance in the treatment of burns and skin ulcers, including neuropathic foot ulcers, venous ulcers, and pressure ulcers. See the extensive section on Skin Transplantation in Chapter 21.

PROGNOSIS.

Most clients have multiple complicating medical factors that contribute to poor wound closure. Each client responds differently to a course of therapy. Provided there is no infection, there is a good blood supply, the pressure has been eliminated or redistributed, and the client is not malnourished and has no medical complications, the wound should heal successfully. The presence of any of these factors alters the prognosis negatively.

10-17 SPECIAL IMPLICATIONS FOR THE THERAPIST

Pressure Ulcers

PREFERRED PRACTICE PATTERNS

7B:

Impaired Integumentary Integrity Associated with Superficial Skin Involvement

7C:

Impaired Integumentary Integrity Associated with Partial-Thickness Skin Involvement and Scar Formation

7D:

Impaired Integumentary Integrity Associated with Full-Thickness Skin Involvement and Scar Formation

7E:

Impaired Integumentary Integrity Associated with Skin Involvement Extending into Fascia, Muscle, or Bone and Scar Formation

The therapist plays a pivotal role in the prevention and management of pressure ulcers. Not only is the therapist an expert in the delivery of therapeutic modalities, but also appropriate positioning, management of tissue load (mechanical factors acting on the tissues), and good mobility are essential to the success of the intervention.

High-risk clients should be identified using the Braden Scale or Norton Scale (see Tables 10-10 and 10-11), but all clients in the health care delivery system should be evaluated for risk levels and reassessed at least every 3 months for changes in status (or when there is a change in medical status).

Anyone with a history of previous pressure ulcer is considered high risk requiring a prevention protocol immediately. Acute care clients should be reassessed daily or at least weekly, on transfer to another unit/floor, and with changes in medical status.

The high-risk client will need frequent position changes, at least every 2 hours in bed, and at least every hour while sitting, every 15 minutes if the client can move himself or herself. Utilizing all turning surfaces, position the client at a 35-degree oblique angle when side lying (Fig. 10-37).

Figure 10-37 Following the “rule of 30s” (head position at 30 degrees; see Fig. 10-38) and bed positioning should include side-lying at oblique angles, usually described as a 30-to 45-degree side-lying position to either side. (Courtesy National Pressure Ulcer Advisory Panel, 2000.)

Elevate the head of the bed to no greater than 30 degrees when the client is supine (Fig. 10-38); if the head of the bed is elevated beyond 30 degrees (e.g., for eating, watching television, nursing care, or therapy intervention) the duration of this position needs to be limited to minimize both pressure and shear forces. A trapeze bar, turning sheet, or transfer board can be used to prevent shearing injury to the skin during movement or position change. Frequent shifting of body weight prevents ischemia by redistributing the weight and allowing blood to recirculate.

Figure 10-38 Head of bed at 30 degrees. Using a goniometer to determine a 30-degree angle, the therapist can mark the bed frame and raised headboard with colored tape to signify maximum inclination for pressure ulcer prevention. Client, family, and staff education and cooperation are vitally important and remain key intervention strategies. (Courtesy Anthony Stabler, Byron Health Center, Fort Wayne, IN. National Pressure Ulcer Advisory Panel, 2000.)

Static or dynamic pressure-redistribution devices using air, gel or water, foam, or other substances are commercially available, but the therapist must be aware that the material covering these devices can also create heat and friction contributing to pressure. Redistributing pressure on the skin must be accompanied by adequate fluid and nutrition intake (see also Box 10-16). Doughnut cushions should never be used, because they can cause tissue ischemia and new pressure ulcers.

The person who is incontinent presents an additional challenge to keeping the skin clean and dry. Stool or urine becomes an irritant and places the person at additional risk for skin breakdown. Contamination of an already existing wound by wound drainage, perspiration, urine, or feces is also a concern for the incontinent and immobile population.

Fecal containment products are available for use in the case of acute diarrhea or fecal incontinence when these conditions contribute to the development of ulcers. New products for urinary or fecal incontinence are available to help prevent skin maceration from backflow of urine or feces. These include skin barriers, ointments, and fecal incontinence systems.

Cleaning should be carried out using a mild agent that minimizes irritation and dryness of the skin. Avoid harsh alkali soaps, alcohol-based products that can cause vasoconstriction, tincture of benzoin (may cause painful erosions), and hexachlorophene (may irritate the CNS). During cleaning or wound care, the force and friction applied to the skin should be minimized. Ideal is the use of a disposable, no-rinse, perineal cloth that is impregnated with a barrier ingredient.

Anyone performing PLWS (see Fig. 10-32) must be aware of the potential for aerosolization of microorganisms from a wound during this intervention. Therapists and others in the room during PLWS should wear appropriate personal protective equipment to limit contact with infectious agents. To prevent possible exposure of other clients, PLWS should be performed in a private room or in a treatment room with walls and doors that close, not curtains.⁸⁸

In 2004 and 2005 the FDA and CDC made recommendations for infection control that included the aforementioned plus cover any exposed supplies or client items in the room, cover any open areas not being treated (tubes, ports, etc), consider masking the patient, observe Standard Precautions, dispose of disposables in appropriate waste stream, discard suction canister or liner after each treatment, do not reuse single-use-only items, and after treatment disinfect thoroughly all environmental surfaces.^45,92

Concerns that high-pressure (output pressure of 70 psi) lavage may disseminate contaminants to surrounding tissues have not been substantiated. Until further research establishes safe levels of irrigation pressure in wound cleansing, therapists are advised to use the AHCPR guideline of irrigation pressures between 4 and 15 psi.⁸⁹ All wound management PLWS products provide a psi of 15 or less.

Pigmentary Disorders

Definition and Overview

Skin color or pigmentation is determined by the deposition of melanin, a dark polymer found in the skin, as well as in the hair, ciliary body, choroid of the eye, pigment layer of the retina, and certain nerve cells.

Melanin is formed in the melanocytes in the basal layer of the epidermis and is regulated (dispersion and aggregation) through the release of melatonin, a pineal hormone.

Hyperpigmentation is the abnormally increased pigmentation resulting from increased melanin production. Hypopigmentation is the abnormally decreased pigmentation resulting from decreased melanin production.

Pigmentary disorders (either hyperpigmentation or hypopigmentation) may be primary or secondary. Secondary pigmentary changes occur as a result of damage to the skin, such as irritation, allergy, infection, excoriation, burns, or dermatologic therapy, such as curettage, dermabrasion, chemical peels, or freezing with liquid nitrogen.

Etiologic and Risk Factors

The formation and deposition of melanin can be affected by external influences such as exposure to heat, trauma, solar or ionizing radiation, heavy metals, and changes in oxygen potential. These influences can result in hyperpigmentation, hypopigmentation, or both. Local trauma may destroy melanocytes temporarily or permanently, causing hypopigmentation, sometimes with hyperpigmentation in surrounding skin.

Other pigmentary disorders may occur from exposure to exogenous pigments, such as carotene, certain metals, and tattooing inks. Carotenemia occurs as a result of excessive carotene in the blood, usually from ingesting certain foods (e.g., carrots, yellow fruit, egg yolk). It may also occur in diabetes mellitus and in hypothyroidism. Exposure to metals such as silver can cause argyria, a poisoning marked by a permanent ashen grey discoloration of the skin, conjunctivae, and internal organs. Gold, when given long term for rheumatoid arthritis, can also cause pigmentary changes.

Clinical Manifestations

Hyperpigmentation.: Primary disorders in the hyperpigmentation category include pigmented nevi, mongolian spots, juvenile freckles (ephelides), lentigines (also called liver spots) from sun exposure, café au lait spots associated with neurofibromatosis, and hypermelanosis caused by increased melanocyte-stimulating hormone (e.g., Addison’s disease).

Secondary hyperpigmentation most commonly occurs after another dermatologic condition, such as acne (e.g., postinflammatory hyperpigmentation seen in dark-skinned people). Melasma, a patterned hyperpigmentation of the face, can occur as a result of steroid hormones, estrogens, and progesterones, such as occurs during pregnancy and in 30% to 50% of women taking oral contraceptives. Secondary hyperpigmentation may also develop as a phototoxic reaction to medications, oils in perfumes, and chemicals in the rinds of limes, other citrus fruits, and celery.

Hypopigmentation and Depigmentation.: The disorder most commonly seen by a therapist in the hypopigmentation/depigmentation category is vitiligo. In vitiligo, pigment cells (melanocytes) are destroyed resulting in small or large circumscribed areas of depigmentation often having hyperpigmented borders and enlarging slowly (Fig. 10-39). This condition may be associated with hyperthyroidism, hypothyroidism, pernicious anemia, diabetes mellitus, Addison’s disease, and carcinoma of the stomach.

Figure 10-39 Vitiligo is a term derived from the Greek word for “calf” used to describe patches of light skin caused by loss of epidermal melanocytes. Note the patchy loss of pigment on the face, trunk, and axilla. This condition can affect any part of the face, hands, or body and can be very disfiguring, especially in dark-skinned individuals. (From Swartz MH: Textbook of physical diagnosis, ed 5, Philadelphia, 2006, Saunders.)

Hypopigmentation can also occur on African American skin from the use of liquid nitrogen. Intraarticular injections of high concentrations of corticosteroids may also cause localized temporary hypopigmentation.

Blistering Diseases

Definition, Incidence, and Etiologic Factors

On occasion, blistering diseases may be seen in a therapy practice when severe enough to warrant localized treatment intervention (wound management). Blisters occur on skin and mucous membranes in a condition called pemphigus, which is an uncommon intraepidermal blistering disease in which the epidermal cells separate from one another. This disease occurs almost exclusively in middle-aged or older adults of all races and ethnic groups.

The exact cause of blistering diseases is unknown, but they may occur as a secondary event associated with viral or bacterial infections of the skin (e.g., herpes simplex, impetigo) or local injury of the skin (e.g., burns, ischemia, dermatitis), or they may be drug induced (e.g., penicillamine, captopril). In other diseases, blistering of the skin occurs as a primary autoimmune event characterized by the presence of autoantibodies directed against specific adhesion molecules of the skin and mucous membranes.³⁷

Paraneoplastic pemphigus, an autoantibody-mediated mucocutaneous disease associated with underlying neoplasm, is a syndrome that has a distinct clinical and histologic presentation. This form of pemphigus has a poor prognosis because of the underlying malignancy.

Clinical Manifestations

Blistering diseases are characterized by the formation of flaccid bullae, or blisters. These bullae appear spontane- ously, often on the oral mucous membranes or scalp, and are relatively asymptomatic. Erosions and crusts may develop over the blisters, causing toxemia and a mousy odor. The lesions become extensive, and the complications of the disease, especially infection, can lead to great toxicity and debility. Disturbances of electrolyte balance are also common because of fluid losses through the involved skin in severe cases. See the section on Fluid and Electrolyte Balance in Chapter 5.

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Etiologic and Risk Factors

Pathogenesis

Clinical Manifestations

MEDICAL MANAGEMENT

TREATMENT.

PROGNOSIS.

Lupus Erythematosus

Cutaneous Lupus Erythematosus

MEDICAL MANAGEMENT

PROGNOSIS.

Systemic Sclerosis

Incidence

Etiologic and Risk Factors

Pathogenesis

Clinical Manifestations

MEDICAL MANAGEMENT

TREATMENT.

PROGNOSIS.

Polymyositis and Dermatomyositis

Incidence

Etiologic Factors

Pathogenesis

Clinical Manifestations

MEDICAL MANAGEMENT

TREATMENT.

PROGNOSIS.

THERMAL INJURIES

Incidence and Etiologic and Risk Factors

Pathogenesis and Clinical Manifestations

MEDICAL MANAGEMENT

PROGNOSIS.

Burns

Incidence

Etiologic Factors

Risk Factors

Pathogenesis

Clinical Manifestations

MEDICAL MANAGEMENT

PROGNOSIS.

MISCELLANEOUS INTEGUMENTARY DISORDERS

Pressure Ulcers

Incidence

Etiologic and Risk Factors

Pathogenesis

Clinical Manifestations

MEDICAL MANAGEMENT

TREATMENT.

PROGNOSIS.

Pigmentary Disorders

Etiologic and Risk Factors

Clinical Manifestations

Blistering Diseases

Clinical Manifestations

MEDICAL MANAGEMENT

Cutaneous Sarcoidosis

References