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[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
- 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)
Diagnosis[edit | edit source]
Treatment[edit | edit source]
There is currently no cure. Treatment depends of the symptoms, but may include medication and surgery.
NIH genetic and rare disease info[edit source]
Diffuse cutaneous systemic sclerosis is a rare disease.
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Contributors: Deepika vegiraju
