Martins Paulo Henrique Teixeira, Dallagnese Gabriela, Luzzatto Laura, Dantas Manuela Lima
Department of Dermatology, Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brazil; Department of Dermatology, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
Department of Dermatology, Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brazil; Department of Dermatology, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
An Bras Dermatol. 2019 Sep-Oct;94(5):615-617. doi: 10.1016/j.abd.2019.09.021. Epub 2019 Sep 30.
Langerhans cell histiocytosis is a rare clonal proliferative disease, characterized by the infiltration of one or multiple organs by histiocytes. Due to the diversity of signs and symptoms, the diagnosis of this disease is often late. The estimated incidence in adults is one to two cases per million, but the disease is probably underdiagnosed in this population. This report presents a case of disseminated Langerhans cell histiocytosis. The authors highlight the most characteristic aspects of this rare and heterogeneous disease, which usually presents as a challenging clinical diagnosis.
朗格汉斯细胞组织细胞增多症是一种罕见的克隆增殖性疾病，其特征是组织细胞浸润一个或多个器官。由于体征和症状的多样性，该疾病的诊断往往较晚。成人的估计发病率为每百万人口中有1至2例，但该疾病在这一人群中可能存在诊断不足的情况。本报告介绍了一例播散性朗格汉斯细胞组织细胞增多症病例。作者强调了这种罕见且异质性疾病最具特征性的方面，该疾病通常表现为具有挑战性的临床诊断。
