systemic scleroderma


scleroderma

 [skle″ro-der´mah] chronic hardening and shrinking of the connective tissues of any organ of the body, including the skin, heart, esophagus, kidney, or lung. The skin may be thickened, hard, and rigid, and pigmented patches may occur. The two main types are systemic scleroderma and localized scleroderma. The milder forms are most often seen in women between the ages of 30 and 50; the more severe forms usually affect men, blacks, and older persons. The cause of scleroderma is not known. Current theories suggest three possibilities: an immunological reaction in which the skin attracts lymph cells which stimulate the production of collagen, hereditary factors related to abnormal serum proteins and antinuclear factors, and occupational exposure to silica dust.

Because the symptoms of scleroderma often mimic those of other diseases such as bursitis, osteoarthritis, rheumatoid arthritis, and other collagen disorders, it is difficult to diagnose. There is no test specific for confirmation of a diagnosis of scleroderma, but x-rays, skin biopsies, and tests for antinuclear antibodies, gamma globulin, sedimentation rate, and latex fixation can provide evidence that the disease is present.Treatment. Treatment includes drugs such as penicillamine, immunosuppressives, and antiinflammatory agents. Ulcers that form on the knuckles, elbows, and other bony prominences as a result of calcium deposits are treated topically with ointments. Physiotherapy to restore and maintain musculoskeletal function as much as possible is indicated for certain patients. For others, diet therapy and small frequent feedings can help relieve the problems of malabsorption due to intestinal involvement.Patient Care. Patients with scleroderma have especially thick skin that is hard to penetrate with a needle. Injections and venipunctures can be quite painful and should be done with as much care and dispatch as possible. The site of venipuncture for intravenous therapy is monitored frequently, especially for infiltration, which can cause a painful swelling and pressure for weeks. The skin should be protected from irritation that could lead to breakdown and the introduction of infectious agents.

Calcium deposit sites are assessed regularly to note any signs of prolonged pressure, ulceration, or infection. Cool, moist compresses and a topical ointment can help relieve infections when they do develop at these sites. Protection of the respiratory tract includes stopping smoking, avoiding infectious agents, and getting adequate rest. Emotional stress can aggravate the condition; hence patients are taught effective coping strategies and relaxation techniques.
Mouth care and proper brushing and flossing, as well as periodic dental checks, are necessary to prevent cavities and periodontal disease. Patients with scleroderma have great difficulty opening their mouths wide enough to allow dental work to be done. Patients are encouraged to take prescribed medications exactly as ordered and are taught the names of their drugs, their expected action, and when and how to take them, that is, with meals and never with milk products.
Patients and their families can participate better in self-care and management of the illness if they are provided with continued support, either with a group or by a visiting nurse. Additional information about the disease can be obtained by contacting the United Scleroderma Foundation, P.O. Box 399, Watsonville, CA 95077-0399, telephone (800) 722-HOPE.circumscribed scleroderma 1. localized scleroderma.2. morphea.diffuse scleroderma (generalized scleroderma) systemic scleroderma.linear scleroderma localized scleroderma in a bandlike lesion with discoloration of the skin and atrophy of skin, subcutaneous tissue, muscle, and bone. See also coup de sabre" >coup de sabre. Called also linear morphea.localized scleroderma 1. scleroderma confined to the skin and subcutaneous tissue or secondarily involving the musculoskeletal system. It occurs in three forms: morphea" >morphea, scleroderma" >linear scleroderma, and coup de sabre" >coup de sabre. Called also circumscribed scleroderma.2. morphea.systemic scleroderma a systemic disorder of the connective tissue characterized by hardening and thickening of the skin, with telangiectasias, Raynaud's phenomenon, and fibrotic degenerative changes in organs such as the heart, lungs, kidneys, and gastrointestinal tract. It may remain confined to the face and hands for a long time or may spread diffusely to become generalized. Called also diffuse or generalized scleroderma and systemic sclerosis. See also CREST syndrome.

scle·ro·der·ma

(sklē'rō-der'mă), Do not confuse this word with scleredema or sclerema.Thickening and induration of the skin caused by new collagen formation, with atrophy of pilosebaceous follicles; either a manifestation of progressive systemic sclerosis or localized (morphea). See: systemic sclerosis, morphea. Synonym(s): systemic scleroderma, systemic sclerosis (2) [sclero- + G. derma, skin]

systemic scleroderma

(1) CREST syndrome.
(2) Progressive systemic sclerosis.

scle·ro·der·ma

(sklerō-dĕrmă) Thickening and induration of the skin caused by new collagen formation, with atrophy of pilosebaceous follicles; either a manifestation of progressive systemic sclerosis or localized (morphea).
Synonym(s): dermatosclerosis, systemic scleroderma. [sclero- + G. derma, skin]