Fast Five Quiz: Presentation and Diagnosis of Systemic Sclerosis

Dirk M. Elston, MD

Disclosures

October 09, 2019

Figure 2. Raynaud phenomenon on fingers.

dSSc is characterized by Raynaud phenomenon, which precedes the development of skin changes by about 1 year. dSSc affects the skin proximal to the elbows and knees, the trunk, and the face and may affect many body systems other than the skin (eg, the vascular musculoskeletal, respiratory, cardiovascular, renal, genitourinary, and endocrine systems; the gastrointestinal tract; and the central nervous system).

Telangiectasia of the skin, late involvement of the lungs, and cutaneous calcification (which is variable and rarely results in the rapid development of tumoral calcinosis) are features indicative of lSSc. lSSc affects skin distal to the elbows and knees but can also affect the face and neck. CREST is an acronym used to describe a subset of conditions associated with limited lSSc: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.

Other features of ISSc include:

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

  • Late development of pulmonary hypertension

  • Anticentromere antibodies in approximately 70%-80% of patients

Learn more about the types, physical manifestations, and clinical presentation of SSc.

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