皮肤Rosai-Dorfman病7例临床病理特点分析

Clinical Manifestations and Histopathological Features of Seven Cases with Cutaneous Rosai-Dorfman Disease

目的对皮肤Rosai-Dorfman病的临床表现、皮损形态特征及组织病理学特点进行分析。方法回顾分析7例皮肤Rosai-Dorfman病患者的临床表现、皮损形态特征、组织病理学特点及治疗等。结果 7例患者中男2例,女5例;发病年龄30~66岁。7例患者均为多发皮损。按累及解剖部位分为单处4例,多处3例,共计23处皮损。皮损表现为丘疹结节型4处(17.39%),浸润斑块型14处(60.87%),肿瘤样型5处(21.74%)。7例患者中,皮损单纯表现为丘疹结节型1例,浸润斑块型4例,肿瘤样型1例,1例表现为浸润斑块型及肿瘤样型的混合型。7例患者组织病理表现大致相同,为真皮内混合细胞结节样、灶状或弥漫性浸润,浸润细胞以组织细胞为主,也可见较多的淋巴细胞、浆细胞。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S-100阳性、CD68阳性、CD1a阴性。结论皮肤Rosai-Dorfman 病具有一定的临床和组织病理学特点,皮肤科医生应该提高对该病的认识,以减少误诊和误治。

Objective To explore the clinical and pathological characteristics of cutaneous Rosai-Dorfman disease.Methods Clinical manifestations,morphological features of skin lesions,histopathologic characteristics and treatments of seven patients with cutaneous Rosai-Dorfman admitted to the PLA General Hospital between June 2015 and July 2018 were retrospectively analyzed.Results Two male and five female patients with age ranged from 30 to 66 years were included in this study. All patients presented with multiple skin lesions.Skin lesions involved single anatomical site was seen in four patients and multiple anatomical sites in three patients.Clinically,23 skin lesions in seven patients were divided into three main types:infiltrating plaque type(60.87%),tumor-like type(21.74%)and papulonodular type(17.39%).One case had papulonodular-type lesions,four cases had infiltrating plaque-type lesions,one case had tumor-like lesions,one case had papulonodular-type lesions and infiltrating plaque-type lesions.The histopathological features were similar.They were characterized by a dense or scattered cellular infiltrate containing histocyte,lymphocytes and plasma cells involving the dermis and sometimes the subcutis.The cytoplasm of the histiocytes appeared to contain lymphocytes,neutrophilic granulocytes,a feature known as emperipolesis.These histiocytes were S-100,CD68 positive and CD1a negative.Conclusion Cutaneous Rosai-Dorfman's disease has certain clinical and pathological features.Dermatologists should improve the understanding of the disease to reduce misdiagnosis and mistreatment.