Scleromyxedema: clinical diagnosis and autopsy findings

AUTOR(ES)

Sala, Ana Carolina Bulhões, Cunha, Paulo Rowilson, Pinto, Clóvis Antônio Lopes, Alves, Célia Antônia Xavier de Moraes, Paiva, Ingrid Barreto, Araujo, Ana Paula Vieira

FONTE

An. Bras. Dermatol.

DATA DE PUBLICAÇÃO

2016-10

RESUMO

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.

Documentos Relacionados

• Discrepancies between clinical diagnoses and autopsy findings
• Posterior Circulation Disease. Clinical Findings, Diagnosis and Management
• Autopsy Diagnosis and Technique
• UNEXPECTED AUTOPSY FINDINGS IN UNEXPECTED DEATHS*
• Death of the teaching autopsy: Autopsy findings are important to all clinicians, including general practitioners