Frade Ana Paula, Godinho Mariana Marteleto, Batalha Anna Beatriz Willemes, Bueno Ana Paula Silva
Secretaria Municipal de Saúde do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.
Private Clinic, Rio de Janeiro (RJ), Brazil.
An Bras Dermatol. 2017;92(5 Suppl 1):40-42. doi: 10.1590/abd1806-4841.20175308.
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.
朗格汉斯细胞组织细胞增多症较为罕见，在儿童中更为常见。50%的病例皮肤会受到影响，10%的病例皮肤是唯一受累部位。其病程从自限性和局限性形式到严重的多系统形式不等。先天性病例通常仅累及皮肤且为自限性，数月内可自发缓解。本研究报告了一例罕见的先天性病例，最初局限于皮肤，随后出现播散并导致死亡。一名男婴出生时即有先天性播散性红斑鳞屑性皮损。活检确诊为朗格汉斯细胞组织细胞增多症。根据LCH - 2009方案，患者在数周内发展为多系统形式并开始化疗；然而，患者对治疗无效，最终死亡。
