Thursday, August 12, 2010

Systemic Sclerosis

(telangiectasia pictured left)

Systemic Sclerosis (aka scleroderma) is an autoimmune condition affecting the skin and internal organs. There are 2 forms of Systemic Sclerosis outlined below - but there are also varieties limited to the skin :






        • Diffuse Cutaneous Systemic Sclerosis: as the name implies, there are diffuse skin manifestations, including the torso, arms, legs, face.

        • Limited Cutaneous Systemic Sclerosis. skin involvement is usually limited to the hands, feet and face. We often talk about the CREST syndrome - this is seen in the Limited Cutaneous Systemic Sclerosis.


        CREST Syndrome:

        • Calcinosis: calcium deposits in soft tissue.
        • Raynaud's phenomenon: with vasospasm the digits first turn white, then blue, then a red hyperemic reaction.
        • Esophageal dysmotility.
        • Sclerodactyly: tightening of the skin around fingers and toes, and eventual loss of subcutaneous tissue.
        • Telangiectasia: dilated blood vessels which blanch with pressure.



        Here is a good link to a review on Scleroderma.




        (sclerodactyly pictured left)







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