How is the ISSc type of systemic sclerosis (SSc) characterized?

Updated: Mar 17, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Answer

lSSc is characterized by sclerotic changes of the hands, face, feet, and forearms in addition to the following features (note the images below):

  • Atrophic changes of the ala nasi and lips, facial amimia

  • Telangiectasia of the skin

  • Late involvement of the lungs and late development of pulmonary hypertension

  • Anticentromere antibodies in approximately 70-80% of patients

  • Dilated capillary loops in nail folds: Either videocapillaroscopy or dermatoscopy can be used, with capillary dilatation, giant capillaries, and disrupted vascular configuration visualized. [22] Video capillaroscopy can be performed sequentially and may be useful to detect organ progression. [23] Dermatoscopy is efficient enough to delineate the pathognomonic alterations of the nailfold capillary pattern. It should be performed on all fingers except the thumbs. [24]

  • Cutaneous calcification: This finding varies and may rarely result in the rapid development of tumoral calcinosis. [25] Calcinosis cutis may become widespread. [26]

  • In systemic sclerosis, ulceration at the tip of th In systemic sclerosis, ulceration at the tip of the finger is regarded to be secondary to ischemia.
    Hand of a woman with scleroderma of several years' Hand of a woman with scleroderma of several years' duration: The thickened, tight, thin skin over the fingers is the result of self-amputation of the distal phalanx due to ischemia. Moderately severe flexion contractures of the fingers are present.

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