What are the types of systemic sclerosis (SSc), and how is the dSSc type characterized?

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

Systemic sclerosis is divided into 5 forms: (1) dSSc, (2) lSSc, (3) transitory form (dSSc/lSSc), (4) systemic scleroderma sine scleroderma, and (5) malignant scleroderma. The principal forms are dSSc and lSSc. In addition to the features noted below, dSSc is characterized by Raynaud phenomenon that precedes the development of skin changes by approximately 1 year. Raynaud phenomenon occurs in almost all patients with systemic sclerosis, often as the earliest clinical sign. [19] Note the following:

  • Generalized skin fibrosis of the chest and limbs

  • Areas of skin hyperpigmentation and hypopigmentation (see image below)

    In systemic sclerosis, skin hyperpigmentation of t In systemic sclerosis, skin hyperpigmentation of the lower legs is surrounded by areas of hypopigmentation. The result is a salt-and-pepper appearance.
  • Tendon friction rubs

  • Early involvement of the lungs, kidneys, digestive system, and heart

  • Antibodies against topoisomerase I DNA (Scl 70) in approximately 30% of patients [20, 21]

  • Nail-fold capillary dilatation and capillary destruction (see image below)

    Raynaud phenomenon of the hands: Symmetrical acral Raynaud phenomenon of the hands: Symmetrical acral vasospasm is present, with characteristic pallor, cyanosis, suffusion, and a sense of fullness and tautness.

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