Congenital Langerhans cell histiocytosis: a good prognosis disease?
An. bras. dermatol
;
92(5,supl.1): 40-42, 2017. graf
Artículo
en Inglés
| LILACS
| ID: biblio-887063
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.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Histiocitosis de Células no Langerhans
/
Eritema
Tipo de estudio:
Guía de Práctica Clínica
/
Estudio pronóstico
Límite:
Humanos
/
Masculino
/
Recién Nacido
Idioma:
Inglés
Revista:
An. bras. dermatol
Asunto de la revista:
Dermatologia
Año:
2017
Tipo del documento:
Artículo
País de afiliación:
Brasil
Institución/País de afiliación:
Secretaria Municipal de Saúde do Rio de Janeiro/BR
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