Scleroderma

Progressive systemic sclerosis; Systemic sclerosis; Limited scleroderma; CREST syndrome; Localized scleroderma; Morphea - linear; Raynaud's phenomenon - scleroderma

Scleroderma is a disease that involves the buildup of scar-like tissue in the skin and elsewhere in the body. It also damages the cells that line the walls of the small arteries.

Raynaud's phenomenon

Raynaud's phenomenon is characterized by blood vessel spasms in the fingers, toes, ears or nose, usually brought on by exposure to cold. Raynaud's phenomenon and Raynaud's disease, a similar disorder, may be associated with autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus and scleroderma.

CREST syndrome

The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis (scleroderma). CREST is an acronym for the clinical features that are seen in a patient with this disease. The “C” stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The “R”, stands for Raynaud’s phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The “E” represents esophageal dysmotility, which can cause difficulty in swallowing. The “S” is for sclerodactyly, tightening of the skin causing the fingers to bend. Finally, the letter “T” is for telangiectasia, dilated vessels on the skin of the fingers, face, or inside of the mouth.  Usually only 2 of the five symptoms of the CREST syndrome is necessary to be diagnosed with the disease.

Sclerodactyly

The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.

Telangiectasia

Telangiectasia is the dilation of small superficial vessels and capillaries that cause numerous flat red marks on the hands, face and tongue. Telangiectasia can be a symptom of scleroderma or other systemic diseases.

Causes

Symptoms

Exams and Tests

Treatment

Support Groups

Outlook (Prognosis)

When to Contact a Medical Professional

Prevention