Diffuse cutaneous systemic sclerosis

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Disease Overview

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. 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.[12995][12996] The exact cause of the condition is unknown. There is currently no cure. Treatment depends of the symptoms, but may include medication and surgery.[12995]


Synonyms

  • Diffuse cutaneous systemic sclerosis
  • DcSSc
  • Progressive cutaneous systemic sclerosis
  • Progressive cutaneous systemic scleroderma
  • Diffuse cutaneous systemic scleroderma

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National Organization for Rare Disorders