Zerbini Maria Claudia Nogueira, Sotto Mirian Nacagami, de Campos Fernando Peixoto Ferraz, Abdo Andre Neder Ramires, Pereira Juliana, Sanches José Antônio, Martins Jade Cury
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 .
Autops Case Rep. 2016 Jun 30;6(2):33-8. doi: 10.4322/acr.2016.038. eCollection 2016 Apr-Jun.
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.
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。
