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Article
May 1990

Fibrotic Skin DiseasesClinical Presentations, Etiologic Considerations, and Treatment Options

Author Affiliations

Departments of Dermatology and Biochemistry and Molecular Biology; Departments of Medicine and Biochemistry and Molecular Biology Jefferson Medical College 1020 Locust St Philadelphia, PA 19107

Arch Dermatol. 1990;126(5):661-664. doi:10.1001/archderm.1990.01670290105019
Abstract

Tissue fibrosis, characterized by excessive deposition of collagen and other connective tissue components, is the major histopathologic feature of a variety of clinical conditions. Excessive collagen deposition can occur in internal organs as, for example, in the case of pulmonary fibrosis or liver cirrhosis. Skin is often affected by fibrotic processes, and dermal fibrosis is the pathologic hallmark of several acquired and heritable cutaneous disorders1-3 (Table).

The prototype of fibrotic skin diseases is scleroderma, which can be divided into systemic and localized forms.4,5 Progressive systemic sclerosis (PSS) is a generalized connective tissue disorder, the fibrotic processes affecting not only the skin but also the lungs, heart, kidneys, and the gastrointestinal tract. A subset of systemic scleroderma, the CREST syndrome, demonstrates calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. In most instances, visceral involvement in the CREST syndrome is limited or absent, and, consequently, the prognosis of this form is

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