Systemic Sclerosis

By YUSUF YAZICI, MD
Long Island College Hospital and
the Hospital for Special Surgery, New York
DORUK ERKAN, MD
Hospital for Special Surgery, New York
RACHELLE SCOTT, MD
Cornell University
STEPHEN PAGET, MD
Cornell University

This disorder-also known as scleroderma (SD)-affects the skin and internal organs and is characterized by fibrotic arteriosclerosis of peripheral and visceral vasculature, with variable degrees of extracellular matrix accumulation in the skin and viscera. Dysregulation of collagen( synthesis, endothelial cell injury, and abnormal immunity may be responsible.1 SD can be localized or systemic. Localized SD. Localized disease features primary involvement of the skin with minimal, if any, systemic features. The 3 major types of localized SD are morphea, generalized morphea, and linear SD. Morphea (A) is characterized by indurated dermal or subcutaneous plaques. It is most common in young women. Generalized morphea is relatively uncommon; it occurs when a large number of plaques coalesce. The course of morphea is usually benign, and lesions soften over time. Linear SD usually occurs as a single sclerotic lesion on the extremities or the forehead, in which case it is referred to as coup de sabre. A lesion that crosses a joint can restrict motion. Linear SD usually softens with time. Systemic SD. This condition is characterized by both cutaneous and internal organ fibrosis. Disease can be limited or diffuse. In limited SD, primary involvement is on acral skin (hands, forearms, legs, and face) (B). In diffuse SD, the skin on the trunk and proximal portions of the extremities is extensively sclerosed. Sclerodactyly (tightness of the skin on the fingers with atrophy of soft tissues) is a hallmark of diffuse and limited systemic SD. Common findings are telangiectasia, nailfold capillary disorganization, and abnormal pigmentation on the skin of the hands and fingers. Patients with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome (C) usually have anticentromere antibodies. Disease is milder and progresses more slowly.

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