A chronic disorder of connectitive tissue manifested by hardening and thickening of the skin, occurring in a localized form, and as a systematic disease. The condition is also called dermatosclerosis. It is more common in women than in men (3 to 4:1) and is more common in blacks than whites.

The specific cause of scleroderma is unknown. Possible causes include:

• environmental toxin exposure;

• occupational silica exposure;

• vibrational tool use;

• overproduction of collagen;

• primary vessel injury; or

• an immune dysfunction.

It can be classified into systemic and localized forms.

The systemic forms include diffuse systemic scleroisis (progressive systemic sclerosis) and limited systemic sclerosis (the CREST syndrome, consisting of calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, scleroderma and telangiectasis). The localized forms include morphea and linear scleroderma. The condition is characterized by fibrotic, degenerative, and inflammatory changes in the skin, blood vessels, synovium, skeletal muscle, and internal organs. The skin of a person with scleroderma is taut, firm, and edematous, with decreased flexibility. The appearance of the skin may also change and take on a permanent shine and tightness. There may also be associated joint pain, swelling and stiffness, and Raynaud's phenomenon. Gastrointestinal (GI) disturbances, such as heartburn and dysphagia, and respiratory symptoms, such as shortness of breath, may also be seen.

Careful medical history along with physical examination and laboratory tests may be necessary to diagnose the condition. A biopsy of affected skin tissue may be done to confirm the diagnosis. If renal involvement is present, urinalysis may be done. X-rays and pulmonary function tests may also be parts of the diagnostic process. Right heart catheterization as the standard for diagnosis of associated pulmonary hypertension.

There is no known treatment for scleroderma because no specific cause has been identified. Treatment concentrates on symptoms, and preventing secondary complications. The measures may include:

• medications to treat hypertension;

• blood vessel dilators to treat Raynaud's phenomenon;

• analgesics for joint pain;

• antibiotics for intestinal conditions;

• physical therapy to limit stiffening effects and to help maintain blood flow;

• warm gloves worn to protect the hands from exposure to cold; and

• antacids used for manifestations, such as heartburn or to protect the esophagus from reflux.

If the person with scleroderma smokes, cessation of smoking may be recommended because of the effects of nicotine on blood vessels.

Scleroderma is a progressive disease that may affect every organ of the body. Systemic hypertension, pulmonary hypertension, renal crisis cardiomyopathy, and intestinal tract problems are just some of the possible complications. These complications may be life threatening.