Diffuse cutaneous systemic sclerosis

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

The exact cause of the condition is unknown.

Signs and symptoms

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

• Autoimmunity(Autoimmune disease)
• Dyspnea(Trouble breathing)
• Gastroesophageal reflux(Acid reflux)
• Narrow foramen obturatorium
• Oliguria
• Pulmonary infiltrates(Lung infiltrates)

30%-79% of people have these symptoms

• Arthralgia(Joint pain)
• Arthritis(Joint inflammation)
• Carious teeth(Dental cavities)
• Dyspareunia
• Dysphagia(Poor swallowing)
• Flexion contracture(Flexed joint that cannot be straightened)
• Malabsorption(Intestinal malabsorption)
• Muscle weakness(Muscular weakness)
• Osteolysis(Breakdown of bone)
• Pulmonary fibrosis
• Skin ulcer(Open skin sore)
• Telangiectasia of the skin
• Xerostomia(Dry mouth)

5%-29% of people have these symptoms

• Abnormal bowel sounds
• Congestive heart failure(Cardiac failure)
• Hypertensive crisis
• Nausea and vomiting
• Pulmonary arterial hypertension(Increased blood pressure in blood vessels of lungs)
• Renal insufficiency(Renal failure)

Treatment

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

NIH genetic and rare disease info

• NIH info on Diffuse cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis is a rare disease.

Diffuse cutaneous systemic sclerosis Resources
Need Help Finding a Doctor? Need help finding a doctor or specialist anywhere in the world? Explore our world directory. WikiMD's DocFinder can assist you in locating millions of physicians.	Medical Knowledge Access the latest research - Most recent articles Ongoing trials.

Translate to: East Asian 中文, 日本, 한국어, South Asian हिन्दी, Urdu, বাংলা, తెలుగు, தமிழ், ಕನ್ನಡ,
Southeast Asian Indonesian, Vietnamese, Thai, မြန်မာဘာသာ, European español, Deutsch, français, русский, português do Brasil, Italian, polski