Scleromyxedema: clinical diagnosis and autopsy findings
An. bras. dermatol
; An. bras. dermatol;91(5,supl.1): 48-50, Sept.-Oct. 2016. graf
Article
en En
| LILACS
| ID: biblio-837957
Biblioteca responsable:
BR1.1
ABSTRACT
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
LILACS
Asunto principal:
Piel
/
Escleromixedema
Tipo de estudio:
Diagnostic_studies
Límite:
Humans
/
Male
Idioma:
En
Revista:
An. bras. dermatol
Asunto de la revista:
DERMATOLOGIA
Año:
2016
Tipo del documento:
Article
País de afiliación:
Brasil
Pais de publicación:
Brasil