ACR Codes: 68.613
Scleroderma is a chronic disease of unknown etiology, characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities, especially in the skin and articular structures. Many of the manifestations of this disease have vascular involvement, principally Raynauds phenomenon. The hands and face generally show the most marked changes, especially in the early stages of disease. Patients can also have pruritis, calcinosis, edema, telangiectasias, and sclerodactyly. This disease can range from mild to severe, affecting only the skin or many crucial organs, ultimately causing death if severe. The distribution of skin lesions and the accompanying pattern of internal organ involvement forms the basis for the current classification system of scleroderma into localized and systemic forms of the disease. The organs most commonly affected include GI, renal, pulmonary, and cardiac. This patient has at least three of these. Treatment is generally symptomatic, to involve esophageal dilation, avoiding cold and smoking, skin lotions, ACE-I for hypertensive renal crises, and corticosteroids.
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Disease Topic 4168 
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