Diffuse cutaneous systemic sclerosis

From WikiMD's Wellness Encyclopedia

Other Names: Diffuse cutaneous systemic sclerosis; DcSSc; Progressive cutaneous systemic scleroderma; Progressive cutaneous systemic sclerosis; Diffuse cutaneous systemic scleroderma

A variant of systemic scleroderma characterized by sclerosis of the skin, raynaud phenomenon, and organ involvement, including pulmonary fibrosis, renal disease, and gastrointestinal tract involvement.

Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of systemic scleroderma (systemic sclerosis) characterized by skin hardening (fibrosis) and problems in many organs of the body. The disease can occur at any age but mainly affects people between 40 and 50 years of age.

Cause[edit | edit source]

The exact cause of the condition is unknown.

Signs and symptoms[edit | edit source]

Symptoms include Raynaud’s phenomenon; skin fibrosis beginning on the fingers and face that rapidly becomes generalized; "spider veins" (telangiectasias) on the thorax, face, lips, tongue, and fingers; gastroesophageal reflux; and difficulty eating (dysphagia) along with weight loss, vomiting, diarrhea or constipation. Dry mouth and dental involvement can occur. Joint pain (arthralgias), muscular pain, weakness, cramps, and destruction of the tips of the fingers or toes (acroosteolysis) are frequent. Severe problems involving the lung (fibrosis or pulmonary hypertension) and kidney problems may also occur.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

30%-79% of people have these symptoms

5%-29% of people have these symptoms

Diagnosis[edit | edit source]

Treatment[edit | edit source]

There is currently no cure. Treatment depends of the symptoms, but may include medication and surgery.

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NIH genetic and rare disease info[edit source]

Diffuse cutaneous systemic sclerosis is a rare disease.


Diffuse cutaneous systemic sclerosis Resources
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