Scleromyxedema: clinical diagnosis and autopsy findings
An. bras. dermatol
;
91(5,supl.1): 48-50, Sept.-Oct. 2016. graf
Article
Dans Anglais
| LILACS
| ID: biblio-837957
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.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Peau
/
Scléromyxoedème
Type d'étude:
Etude diagnostique
Limites du sujet:
Humains
/
Mâle
langue:
Anglais
Texte intégral:
An. bras. dermatol
Thème du journal:
Dermatologie
Année:
2016
Type:
Article
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
Universidade Estadual de Campinas/BR
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