Scleroderma

The Autoimmune Disorder

Signs & Symptoms

Skin Symptoms are skin hardness and thickening, skin darker or lighter than normal, stiffness & tightness skin of fingers, hands and forearm, sore on fingertips or toes, tight and mask-like skin on the face, small white lumps under skin that sometimes ooze, hair loss, and fingers or toes turn blue or white in response to hot and cold temperatures

Bone and muscle symptoms include joint pain, numbness and pain in feet, wrist pain, and pain, stiffness, and swelling of fingers and joints

Breathing problems may result from scarring in the lungs and are dry coughs, shortness of breath, and wheezing.

Digestive tract problems are bloating after meals, constipation, diarrhea, difficulty swallowing, heartburn, and problems controlling stool.

Pathogenesis

Scientists are not quite sure what causes the disorder. A build up of collagen in the skin and other organs leads to the symptoms of the disease.

Diagnosis

Medical professionals would do a physical exam, exam may show hard, tight, thick skin. Blood testing, ANA panel, antibody testing, rheumatoid factor, and ESR. Also may be a chest x-ray, CT scan of the lungs, echocardiogram, urinalysis, and skin biopsy.

Management

No specific treatment, doctor will prescribe medicines and other treatments to control your symptoms and prevent complications. Medicines used to treat the disorder are Corticosteroids, nonsteroidal anti-inflammatory drugs, and drugs that suppress the immune system such as methotrexate and Cytoxan. Other treatments are medicines for heartburn, blood pressure medications, light therapy to relieve skin, medicines to improve breathing, and physical therapy.

Prognosis

In some people, symptoms develop quickly at the beginning few years and continue to get worse, but in most people the disease slowly gets worse. People with just the skin symptoms have a better outlook. Widespread scleroderma can lead to cancer, heart failure, scarring of lungs, kidney failure, problems absorbing nutrients from food, and pulmonary hypertension.

Epidemiology

In US over past two decades was a prevalence estimate for adults of 240 per million. More frequent in US than in continental Europe, the United Kingdom, and some areas in Asia.