Scleroderma


What is it?

Scleroderma, also known as systemic sclerosis, is a chronic connective tissue disease characterized by the hardening of skin and connective tissue. There are two classifications of this disorder: localized scleroderma and systemic scleroderma. The localized form is characterized by the limited appearance of symptoms on certain parts of the skin; in the systemic form, symptoms are not localized and can affect the skin, esophagus, gastrointestinal system, lungs, kidneys, heart, and other internal organs.

Who is at risk?

Localized scleroderma occurs most commonly in children, while systemic scleroderma is more common in adults. Though both forms can appear at any age, onset is most frequent between the ages of 25 and 55; the disease also affects three to four times more females than males.

Symptoms

Though scleroderma manifests differently among patients,  the most prominent symptom is hardening of the skin. Other symptoms include, but are not limited to:

  • Calcinosis: formation of white calcium lumps under the skin.
  • Raynaud’s phenomenon: fingers or toes turn white in cold temperatures due to poor circulation.
  • Esophageal dysfunction: difficulty swallowing or heartburn.
  • Sclerodactyly: tightening and hardening of skin of the fingers and toes.
  • Telangiectasia: dilation of blood vessels.

The systemic form scleroderma involves the sclerosis (hardening) of internal organs. Depending on which part of the body is affected, additional symptoms may include:

  • High blood pressure (if the heart is affected)
  • Kidney failure
  • Respiratory problems
  • Carpal tunnel syndrome

Causes

Though the exact cause of scleroderma is yet to be determined, the disease itself involves the overproduction of collagen, a protein that binds skin and connective tissue.

Diagnosis

Diagnosis of scleroderma is based on physical examination, skin assessment tests, blood tests, and biopsies. 

sources: http://www.niams.nih.gov/Health_Info/Scleroderma/scleroderma_ff.asp

http://www.scleroderma.org/site/PageNavigator/patients_whatis.html#.UsGSUdLunDs

 

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