Scleroderma
Scleroderma is an arthritis-related condition that is classified as an autoimmune connective-tissue disease. You may think scleroderma is a single disease, but it's not. Scleroderma is a symptom of a group of diseases that are complicated by the abnormal growth of connective tissue supporting skin and other organs. The term
Scleroderma Types
There are two major types of scleroderma, and those are further classified. The two major types of scleroderma are:
Localized scleroderma — Primarily affects the skin and is further subdivided into morphea and linear. Morphea is characterized by hard, oval patches on the skin. Linear is characterized by a line of thickened, discolored skin commonly on the arms, legs, or forehead.
Systemic sclerosis — Is further subdivided into limited scleroderma, diffuse scleroderma, and sine scleroderma. Limited scleroderma typically has gradual onset, is limited to certain areas of the body, and may affect internal organs eventually. Diffuse scleroderma is characterized by sudden onset, thickening covering a large area of the body, and may also affect internal organs. Sine scleroderma does not affect the skin.
CREST
Systemic sclerosis patients may have a typical pattern of symptoms referred to as CREST. The acronym CREST stands for:
Calcinosis — calcium deposits in connective tissue
Raynaud's phenomenon — small blood-vessel constriction in response to cold or stress
Esophageal dysfunction — muscle in lower esophagus functions improperly
Sclerodactyly — tight, thick, shiny skin on toes and fingers
Telangiectasias — tiny red spots on face and hands
Diagnosing Scleroderma
There is no single test that is used to diagnose scleroderma, although most people with scleroderma are positive for antinuclear antibodies. A physical examination, in combination with blood tests to rule out other conditions, plus telltale symptoms of organ involvement, are all used to formulate a diagnosis.
There is neither a cure for scleroderma, nor a treatment that can prevent the thickening which is characteristic of the disease. While there is no great treatment for the skin, there are reports of benefits with some medications. Medications are usually prescribed for the consequences of scleroderma such as arthritis, pulmonary hypertension, hypertension, heartburn, kidney problems, and more.
Alert
Medications used to treat other autoimmune conditions, including rheumatoid arthritis and lupus, typically have little effect on scleroderma patients. Scleroderma is a somewhat rare disease with only twelve to twenty new cases per million diagnosed each year.
Prevalence of Scleroderma
Approximately 75,000 to 100,000 people in the United States are affected by scleroderma. Women between the ages of thirty and fifty are the most commonly affected group, but men, women, and children can all develop scleroderma.
