Congenital Langerhans cell histiocytosis: a good prognosis disease?
An. bras. dermatol
;
92(5,supl.1): 40-42, 2017. graf
Article
in English
| 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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Histiocytosis, Non-Langerhans-Cell
/
Erythema
Type of study:
Practice guideline
/
Prognostic study
Limits:
Humans
/
Male
/
Infant, Newborn
Language:
English
Journal:
An. bras. dermatol
Journal subject:
Dermatology
Year:
2017
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Secretaria Municipal de Saúde do Rio de Janeiro/BR
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