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A Model for Fibrosis

Raul Fleischmajer, MD; Jerome S. Perlish, PhD; Matthew Duncan, PhD

Arch Dermatol. 1983;119(12):957-962.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Systemic scleroderma or progressive systemic sclerosis is a connective-tissue disease characterized by vascular alterations and severe fibrosis. The disease has a predilection for female patients and affects all races and ages, although it is more common during the third and fourth decades of life. The sites most frequently involved are the skin, esophagus, lung, and kidney. Patients with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) seem to have a more benign clinical course than those with the diffuse form of the disease, which has a more serious prognosis owing to the high incidence of severe pulmonary fibrosis and kidney insufficiency.

The pathophysiology of scleroderma remains poorly understood. Two schools of thought prevail: some believe that the primary event is vascular damage, and others propose a derangement in collagen metabolism as the initial event.¹ More recently, emphasis has been placed on the presence of mononuclear cell infiltrates . . . [Full Text PDF of this Article]

Author Affiliations
Department of Dermatology Mount Sinai School of Medicine 1 Gustave L. Levy PI New York, NY 10029

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