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Scleroderma is a chronic, progressive disorder of connective tissue that takes its name from the Greek "skleros," meaning hard, and "derma," meaning "skin." The thickening and hardening of the skin affects the fingers, and also may involve the hands, face, neck, and trunk. The skin changes may be accompanied by a spectrum of local or general tissue fibrosis and inflammatory infiltrates involving the joints and internal organs (GI tract, lungs, kidneys, heart and blood vessels, as well as the genitourinary tract).
The precise etiology of scleroderma remains unclear. Scleroderma occurs because the body overproduces and accumulates collagen, a type of fibrous protein found in skin, bone, tendon, and cartilage. Increased collagen production may result from immune system dysfunction. Environment factors may cause scleroderma. Some cases have been linked to exposure to solvents, silica, radiation, or certain medications (bleomycin, pentazocine).
When scleroderma involves only the skin and the tissue immediately beneath the skin, it is called localized scleroderma. Localized scleroderma is further subdivided into two types: morphea and linear scleroderma. In morphea (focal cutaneous scleroderma), oval patches appear in limited areas on the skin. In linear scleroderma, bands or streaks of hardened skin appear on the arms, legs, or forehead.
Systemic scleroderma, in contrast to localized, involves the internal organs. CREST syndrome (an acronym for the symptoms) is a type of limited systemic scleroderma that carries a better prognosis because it does not involve the heart, lungs, or kidneys. Diffuse scleroderma (also called progressive systemic sclerosis) usually has a rapid onset and a worse prognosis because it involves the lungs, kidneys, and gastrointestinal tract. Systemic scleroderma occurs less frequently than localized scleroderma.
The usual course of localized scleroderma involves a period of several years during which the skin and underlying tissues harden and stiffen progressively. After that time, the skin symptoms rarely become worse and may even improve. In rare instances, the hands may become permanently debilitated.
The organ involvement active in systemic scleroderma may produce pulmonary hypertension, lung problems, heart disease, kidney failure, and intestinal tract problems. The cause of death is usually kidney failure or pulmonary hypertension.
Incidence and Prevalence: The estimated incidence of systemic scleroderma is 19 cases per million people; estimated prevalence is 240 cases per million. Prevalence has increased due to earlier detection, better diagnostic techniques, and longer survival rates. Determining accurate rates of incidence and prevalence is difficult since misdiagnosis is common. Among women in the US aged 35 to 65 years, the prevalence of systemic scleroderma has been estimated as high as 400 cases per million people (Jimenez). |
Source: Medical Disability Advisor
| Scleroderma does not appear to be hereditary. The disease is 3 to 9 times more common in women than in men and is rare in children. Onset usually occurs in individuals aged 30 to 50 years (Jimenez). Scleroderma occurs in all ethnic groups and nationalities, but some patterns do occur among disease types. Localized scleroderma is more common among individuals of European descent than among those of African descent. Morphea tends to appear in individuals aged 20 to 40. Linear scleroderma is usually found in teenagers and children. Systemic scleroderma affects more women of African descent than of European ("Scleroderma: A Handout on Health"), putting blacks at slightly more risk than whites (Jimenez). |
Source: Medical Disability Advisor
History: The symptoms and signs of localized scleroderma are tightening and hardening of the skin, particularly on the arms, face, or hands, resulting in a loss of flexibility. These areas may also show changes in pigmentation. The individual may report swollen hands and feet (edema), particularly in the morning. Joint pain and stiffness may occur.
Symptoms and signs of systemic scleroderma include those of localized scleroderma. Most individuals complain of cold, numb fingers in response to cold environments, the symptom being caused by spasm of the blood vessels (Raynaud's phenomenon). Muscle and joint pains, fever, and muscle weakness are common. Individuals may report heartburn (gastroesophageal reflux), difficulty swallowing (dysphagia), regurgitation of food, and impaired speech (dysarthria). The individual with gastrointestinal involvement may report dry mouth, weight loss (anorexia), nausea, vomiting, bloating, diarrhea, and constipation. Individuals with advanced scleroderma may report a dry cough, chest pain, or shortness of breath. Physical exam: Scleroderma can be difficult to diagnose because it is uncommon and may resemble other diseases at the beginning (e.g., lupus, rheumatoid arthritis). Skin changes are the hallmark of scleroderma. Thickened, hardened, shiny skin that has lost its normal texture and folds, changes in skin pigmentation, dilated blood vessels (telangiectasia) on the face and hands, and ulcers on the fingertips may be apparent. The joints of the fingers may become fixed and show decreased range of motion. Heart and lung problems may be detected. A grating sound (tendon friction rubs) may be heard or felt as inflamed tissues move over one another at the fingers, wrists, elbows, shoulders, knees, and ankles. Tests: Blood tests for antibodies directed against parts of the cells in an individual’s body (antinuclear antibodies [ANA], anticentromere, and others) may show a pattern consistent with scleroderma. These tests are not helpful in monitoring disease activity. A sample of the affected skin may be removed (biopsy) for analysis. Examination of the junction between the fingernail and the skin under a microscope may reveal fewer small blood vessels (capillaries) than usual. Other diagnostic tests depend on affected organ systems. For instance, in systemic scleroderma, endoscopy may be performed for gastrointestinal involvement, electrocardiogram (ECG) or a 24-hour Holter monitor for heart concerns, and pulmonary function tests for lung involvement. |
Source: Medical Disability Advisor
| No cure exists for scleroderma because there is no way to stop collagen overproduction; however, there are medications to treat some of the symptoms. Marks on the face, created by tiny dilated blood vessels (telangiectasia), may be treated with pulsed dye laser surgery or camouflaged with special makeup. Antihypertensive medications, antiplatelet agents, vasodilators, and aspirin are used to treat systemic sclerosis. Antibiotics are often prescribed to control the overgrowth of intestinal flora. Other gastrointestinal problems are treated with antacids, prokinetic agents to stimulate motility, and dietary changes. Circulatory problems, including Raynaud's phenomenon, may be treated with calcium channel blockers, alpha blockers, angiotensin-converting enzyme (ACE) inhibitors, and low-dose aspirin. Joint pain and stiffness may be eased with aspirin, nonsteroidal anti-inflammatory drugs (NSAIDS), or low-dose corticosteroids. Disease-modifying antirheumatic drugs (DMARDS) and immunosuppressants may be prescribed to help control the immune system. Precautions should be taken to avoid cold exposure, and individuals should stop smoking ("Scleroderma"; Jimenez; "Scleroderma: A Handout on Health"). |
Source: Medical Disability Advisor
In most individuals with localized scleroderma, the disease is self-limited. Hand debilitation occurs to varying degrees.
Systemic scleroderma usually progresses over a period of several years. For individuals with limited systemic involvement, 10-year survival rates are approximately 60% to 70%. For those with wider (diffuse) involvement, 10-year survival rates are about 20% (Jimenez). There may be periods when the disease process is stable. Occasionally, the symptoms may lessen (enter remission) in either localized areas or throughout the body. When the disease involves the lungs or kidneys, it may become life-threatening. Without treatment, systemic scleroderma may be fatal within a few years. Factors implying a more severe prognosis include young age at diagnosis, extensive skin involvement with rapid progression, African descent, anemia, elevated erythrocyte sedimentation rate (ESR), and pulmonary and renal involvement. |
Source: Medical Disability Advisor
Although scleroderma is a chronic, progressive systemic disease, individuals may benefit from a comprehensive rehabilitation program. The goals of rehabilitation are to promote independence in all functional activities while preventing loss of motion and strength (Steen).
Rehabilitation should include general conditioning exercises that emphasize full range of motion and strengthening. Breathing exercises should promote full chest expansion and mobility of the chest wall. Heat in various forms, including paraffin baths (Sandqvist) and hydrotherapy, may relieve symptoms and enhance mobility.
A lifetime program of walking, aquatic exercise, stretching exercises, and other physical fitness programs should be initiated before discharge from therapy.
Occupational therapy may also be necessary to address independence in activities of daily living. A home assessment might be indicated to ascertain that the environment is optimal for the individual's needs. Assistive devices might be recommended to aid with common daily tasks.
An ergonomic evaluation to assess the work station may enable individuals with scleroderma to maintain employment.
Because individuals may become depressed if their activities are restricted due to exacerbations of this disease, counseling or a support group might be needed. Individuals may also benefit from the experience of other individuals with similar needs (Haustein).
For further information about management of this condition and rehabilitation outcome please refer to the article "Musculoskeletal Involvement in Scleroderma" (Pope). |
FREQUENCY OF REHABILITATION VISITS | | Nonsurgical | |
| Physical or Occupational Therapist | | Up to 24 visits within 12 weeks | |
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| The table above represents a range of the usual acceptable number of visits for uncomplicated cases. It provides a framework based on the duration of tissue healing time and standard clinical practice. |
Source: Medical Disability Advisor
| Some complications are limited to individuals with systemic scleroderma. Others occur with either the systemic or localized type. Injury to sclerotic hands can result in wound infection and potential tissue death (necrosis). Smoking and exposure to cold temperatures can trigger attacks of Raynaud's phenomenon. Individuals with gastroesophageal reflux may develop infections of the esophagus. Dental problems can develop due to dry mouth from decreased saliva production (sicca syndrome). Also, as tightening facial skin shrinks and narrows the mouth opening, tooth brushing and flossing become more difficult. Individuals with scleroderma are twice as likely as the general population to develop cancer, especially lung cancer. Scarring, inflammation, and weakening of the heart muscle may develop. Renal crisis occurs in approximately 10% of individuals with scleroderma ("Scleroderma: A Handout on Health"). Depression is common among individuals with any type of scleroderma. |
Source: Medical Disability Advisor
| The wide variety of symptoms present in scleroderma require that work environment accommodations be considered on an individual basis. In general, work requiring fine motor skills may prove difficult, if not impossible, for individuals with hand debilitation. Accommodations would involve larger keypads for the phone. Typing is more problematic because voice recognition software still often requires the ability to re-type words that are not correctly transcribed. |
Source: Medical Disability Advisor
| If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case. Regarding diagnosis:
- Has diagnosis of scleroderma been confirmed?
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Has type of scleroderma been determined?
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If diagnosis is uncertain, have conditions with similar symptoms been ruled out?
Regarding treatment:
- Although there is no cure for scleroderma, has treatment helped preserve normal body functions?
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Has drug therapy been effective against specific symptoms?
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Is individual participating in an ongoing, home-based physical therapy program to help maintain flexibility and muscle strength?
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Have complications (e.g., tissue necrosis, Raynaud's phenomenon, esophageal infection) responded to appropriate treatment?
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Has individual been advised to stop smoking? If unable to stop, would individual benefit from enrollment in a community stop-smoking program?
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Have psychological issues, such as depression, been appropriately addressed through counseling or support groups?
Regarding prognosis:
- Is individual compliant with treatment plan? If not, what can be done to enhance compliance?
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Does individual’s age at diagnosis or ethnicity imply a more severe prognosis?
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Does extent of skin involvement imply a more severe prognosis?
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Does individual have a coexisting condition (e.g., heart disease, lung disease, kidney disease, gastrointestinal disease, hypertension, cancer, immune system disorders) that may complicate treatment?
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Source: Medical Disability Advisor
| Cited "Scleroderma." MayoClinic.com. 19 Oct. 2006. Mayo Foundation for Medical Education and Research. 23 Sep. 2008 <http://www.mayoclinic.com/invoke.cfm?objectid=3830E935-0B28-4EE9-85E99C261470826D>. "Scleroderma: A Handout on Health." National Institute of Arthritis and Musculoskeletal and Skin Diseases. Jul. 2006. National Institutes of Health (NIH). 23 Dec. 2008 <http://www.niams.nih.gov/Health_Info/Scleroderma>. Haustein, U. F. "Systemic Sclerosis-Scleroderma." Dermatology Online Journal 8 1 (2002): 3. Jimenez, Sergio, et al. "Scleroderma." eMedicine. Eds. John Varga, et al. 8 Jan. 2007. Medscape. 23 Dec. 2008 <http://emedicine.com/med/topic2076.htm>. Pope, J. E. "Musculoskeletal Involvement in Scleroderma." Rheumatic Disease Clinics of North America 29 2 (2003): 391-408. Sandqvist, G., A. Akesson, and M. Eklund. "Evaluation of Paraffin Bath Treatment in Patients with Systemic Sclerosis." Disability Rehabilitation 26 16 (2004): 981-987. Steen, V. D. "Treatment of Systemic Sclerosis." American Journal of Clinical Dermatology 2 5 (2001): 315-325. |
| General "Medical Topics: Scleroderma." MD Consult. 24 Aug. 2007. Elsevier, Inc. 25 Sep. 2008 <http://home.mdconsult.com>. |
Source: Medical Disability Advisor
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