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Type: Artigo
Title: Scleromyxedema: Clinical Diagnosis And Autopsy Findings
Author: Bulhoes Sala
Ana Carolina; Cunha
Paulo Rowilson; Lopes Pinto
Clovis Antonio; Xavier de Moraes Alves
Celia Antonia; Paiva
Ingrid Barreto; Vieira Araujo
Ana Paula
Abstract: Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
Subject: Autopsy
Editor: Soc Brasileira Dermatologia
Rio de Janeiro
Citation: Anais Brasileiros De Dermatologia. Soc Brasileira Dermatologia, v. 91, p. 48 - 50, 2016.
Rights: aberto
Identifier DOI: 10.1590/abd1806-4841.20164527
Date Issue: 2016
Appears in Collections:Unicamp - Artigos e Outros Documentos

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