Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the serious type of the disease, can be fatal. The local type of the disease is not serious.

Signs and symptoms

Scleroderma affects the skin, and in serious, life-threatening cases it affects the blood vessels and internal organs. The most evident symptom is the hardening of the skin. There is discoloration of the hands and feet in response to cold. The seriousness of the disease depends on which organs, if any, are affected. If the heart, lungs, or kidneys are affected, the disease is generally fatal. Most patients (>80%) have Raynaud's phenomenon, a vascular sign in the fingers.

Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin.

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening. The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:

• Calcinosis
• Raynaud's syndrome
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia

These five are the major symptoms of the CREST syndrome. Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of sclerodemra. Morphea/linear scleroderma involve isolated patches of hardened skin - there generally is no internal organ involvement.

Therapy

There is no cure for scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Patients often benefit from exposure to heat.

Pathophysiology

The cause of the disease is unknown. The overproduction of collagen is thought to result from an autoimmune dysfunction.

Epidemiology

Scleroderma affects approximately 300,000 people in the United States. It is four times as common in women than in men. Incidence rates are estimated at 2-20 per million per year in the United States.

Patients' advocacy

The Scleroderma Foundation is a leading organization dedicated to raising awareness of the disease and assisting those who are afflicted. Its national spokesperson is Jason Alexander.

External links

• Scleroderma Foundation
• International Scleroderma Network
• Dermatology Lecture Notes