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光疗成功治疗不确定细胞组织细胞增多症。

Indeterminate cell histiocytosis successfully treated with phototherapy.

作者信息

Zerbini Maria Claudia Nogueira, Sotto Mirian Nacagami, de Campos Fernando Peixoto Ferraz, Abdo Andre Neder Ramires, Pereira Juliana, Sanches José Antônio, Martins Jade Cury

机构信息

Department of Pathology - Faculty of Medicine - University of São Paulo, São Paulo/SP - Brazil .

Internal Medicine Division - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .

出版信息

Autops Case Rep. 2016 Jun 30;6(2):33-8. doi: 10.4322/acr.2016.038. eCollection 2016 Apr-Jun.

Abstract

First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome.

摘要

中间细胞组织细胞增多症于1985年首次被描述,是一种罕见的皮肤树突状细胞疾病,临床表现和病程多样。病理基础是不确定细胞(ICs)的增殖,其免疫表型特征为CD1a、CD68阳性,S100弱阳性/局灶性阳性,以及CD207(朗格汉斯蛋白)阴性。作者报告了一例健康老年女性的病例,该患者在18个月的时间里,躯干、颈部、面部和四肢出现了泛发性圆顶状红色皮肤结节。实验室检查和影像学检查排除了内部受累。皮肤活检结果符合IC组织细胞增多症。患者接受了窄谱中波紫外线光疗,短期疗效极佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9624/4982782/a0399b1ac573/autopsy-06-02033-g01.jpg

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