Congenital Langerhans cell histiocytosis: a good prognosis disease?
An. bras. dermatol
; An. bras. dermatol;92(5,supl.1): 40-42, 2017. graf
Article
em En
| LILACS
| ID: biblio-887063
Biblioteca responsável:
BR1.1
ABSTRACT
Abstract Langerhans cell histiocytosis is rare and more frequent in children. The skin is affected in 50% of the cases and is the only site in 10%. Its course varies from self-limited and localized forms to severe multisystemic forms. Congenital cases are usually exclusively cutaneous and self-limited, with spontaneous remission in months. This study presents a rare congenital case, initially restricted to the skin, with subsequent dissemination and fatal outcome. A male newborn presented congenital disseminated erythematous scaly lesions. The biopsy was conclusive for Langerhans cell histiocytosis. The patient evolved into the multisystemic form in weeks, when chemotherapy was started, according to the LCH-2009 protocol; however, the patient was refractory to treatment and died.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Assunto principal:
Histiocitose de Células não Langerhans
/
Eritema
Tipo de estudo:
Guideline
/
Prognostic_studies
Limite:
Humans
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Male
/
Newborn
Idioma:
En
Revista:
An. bras. dermatol
Assunto da revista:
DERMATOLOGIA
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Brasil
País de publicação:
Brasil