中华皮肤科杂志 ›› 2018, Vol. 51 ›› Issue (3): 173-176.doi: 10.3760/cma.j.issn.0412-4030.2018.03.002

• 论著 • 上一篇    下一篇

皮肤Rosai-Dorfman病临床表现及组织病理学特征分析

张德志1,普雄明2,于世荣3,丁媛3,康晓静4   

  1. 1. 新疆维吾尔自治区人民医院
    2. 乌鲁木齐市新疆维吾尔自治区人民医院皮肤科
    3. 新疆维吾尔自治区人民医院皮肤性病科
    4. 乌鲁木齐市 新疆维吾尔自治区人民医院皮肤科
  • 收稿日期:2017-04-24 修回日期:2017-08-08 出版日期:2018-03-15 发布日期:2018-03-06
  • 通讯作者: 普雄明 E-mail:puxiongming@126.com

Clinical manifestations and histopathological features of cutaneous Rosai-Dorfman disease

  • Received:2017-04-24 Revised:2017-08-08 Online:2018-03-15 Published:2018-03-06

摘要: 目的 探讨皮肤Rosai?Dorfman病(CRDD)的临床表现、皮损形态特征和组织病理学特点。方法 收集20例CRDD患者的基本情况及临床资料,分析其皮损特征并进行分型,同时对皮损进行组织病理学检查和免疫组化染色。结果 20例CRDD患者中,多发皮损11例,单发皮损9例;按累及的解剖部位分单处16例,多处4例,共计24处。皮损表现为丘疹结节型10处(41.67%)、浸润斑块型12处(50.00%)和肿瘤样型2处(8.33%)。20例中6例皮损表现为混合型(丘疹结节型/浸润斑块型5例,浸润斑块型/肿瘤样型1例)。24处组织标本病理表现大致相同,即真皮和(或)皮下脂肪层可见数量不一、体积较大的组织细胞散在或片状分布,伴大量以淋巴细胞和浆细胞为主的炎症细胞浸润,组织细胞胞质内吞噬有数量不一的淋巴细胞、中性粒细胞等。免疫表型均为组织细胞S100阳性、CD68阳性、CD1a阴性。17处皮损病变累及真皮全层,其中13处病变侵入脂肪层;6处病变仅累及真皮浅中层;1处病变仅累及真皮深层及脂肪层。不同形态类型皮损之间的病变累及范围和炎症浸润模式均无明显差异。结论 CRDD临床皮损以丘疹结节型和浸润斑块型为主,肿瘤样型少见。不同形态皮损组织病理均可见数量不等具有伸入运动的组织细胞。S100蛋白、CD68等免疫组化染色有助于CRDD的诊断与鉴别诊断。

关键词: 组织细胞增多症, 窦, 组织细胞, 诊断, 鉴别, 组织病理

Abstract: Zhang Dezhi, Pu Xiongming, Yu Shirong, Ding Yuan, Kang Xiaojing Department of Dermatology and Venereology, People′s Hospital of Xinjiang Uygur Autonomous Region, Urumqi 830001, China Corresponding authors: Pu Xiongming, Email: puxiongming@126.com; Kang Xiaojing, Email: drkangxj666@163.com 【Abstract】 Objective To investigate clinical manifestations, morphological characteristics of skin lesions, and histopathological features of cutaneous Rosai-Dorfman disease (CRDD). Methods Basic information and clinical data were collected from 20 patients with CRDD. According to the morphological characteristics, the skin lesions were classified into different types, and then subjected to histopathological examination and immunohistochemical staining. Results Of the 20 patients with CRDD, 11 had multiple lesions, and 9 had solitary lesions. Skin lesions involved single anatomical site in 16 patients, multiple anatomical sites in 4 patients, and there were a total of 24 involved anatomical sites. Skin lesions on the 24 sites were divided into 3 main types, including papulonodular type (10/24, 41.67%), infiltrating plaque type (12/24, 50.00%) and tumor-like type (2/24, 8.33%). Of the 20 patients, 6 had mixed-type skin lesions, including 5 with papulonodular-type lesions complicated by infiltrating plaque-type lesions, and 1 with infiltrating plaque-type lesions complicated by tumor-like lesions. There were similar histopathological manifestations of skin lesions among the 24 involved anatomical sites. Concretely speaking, varying numbers of large histiocytes were scattered or distributed in sheets in the dermis and/or subcutaneous adipose tissue, with infiltration of plenty of inflammatory cells, mainly lymphocytes and plasma cells. Moreover, varying numbers of lymphocytes and neutrophils were observed in the cytoplasm of histiocytes. Immunohistochemically, these histiocytes were stained positive for S100 and CD68, but negative for CD1a. At 17 anatomical sites, lesions affected the full-thickness dermis, and the subcutaneous adipose tissues were involved at 13 of 17 sites. Of the 24 involved anatomical sites, lesions only affected the superficial to middle dermis at 6 sites, and affected the deep dermis and subcutaneous adipose tissue at 1 site. There were no obvious differences in the extent of lesion involvement and pattern of inflammatory infiltration among different morphological types of skin lesions. Conclusions CRDD mainly manifests as papulonodular-type and infiltrating plaque-type lesions, and tumor-like lesions are rare. Histopathologically, varying numbers of emperipoletic histiocytes can be observed in lesions of different types.

Key words: Histiocytosis, sinus, Histiocytes, Diagnosis, differential, Histopathology