Chu P, LeBoit P E
Department of Dermatology, School of Medicine, University of California, San Francisco 94143-0506.
J Cutan Pathol. 1992 Jun;19(3):201-6. doi: 10.1111/j.1600-0560.1992.tb01659.x.
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. Cutaneous lesions are the most common form of extranodal disease, but cases that present as cutaneous disease without lymph node involvement are rare. We examined biopsies from three patients with cutaneous lesions of sinus histiocytosis, none of whom had detectable systemic involvement, and compared them to those of two patients whose lymph nodes were involved by the disease. The histology of skin lesions in all five cases showed dense nodular or diffuse infiltrates of "histiocytes," emperipolesis of lymphocytes, neutrophils, and plasma cells. "Histiocytes" were present in lumens of dilated lymphatics. At the peripheries of the lesions were lymphoid aggregates and thick-walled vessels surrounded by plasma cells. Staining with anti-S-100 protein antibody showed marked cytoplasmic positivity in the "histiocytes" in each case. The only features that differed among the two groups were the presence of pseudoepitheliomatous hyperplasia and infiltrates of eosinophils in one case in which the disease was limited to the skin. We believe that cutaneous sinus histiocytosis can be specifically diagnosed by skin biopsy. Because cutaneous sinus histiocytosis may be unfamiliar to general pathologists, dermatopathologists, and dermatologists, cases limited to the skin may be under-recognized.
伴巨大淋巴结病的窦组织细胞增生症（罗萨伊 - 多夫曼病）是一种细胞的系统性增殖，这些细胞类似于淋巴结的窦组织细胞。尽管最初的报告强调了常明显的颈部淋巴结病，但超过40%的患者有结外受累。皮肤病变是结外疾病最常见的形式，但以无淋巴结受累的皮肤疾病形式出现的病例很少见。我们检查了3例窦组织细胞增生症皮肤病变患者的活检标本，这些患者均未检测到系统性受累，并将其与2例疾病累及淋巴结的患者的活检标本进行比较。所有5例患者皮肤病变的组织学表现为“组织细胞”的密集结节状或弥漫性浸润、淋巴细胞、中性粒细胞和浆细胞的入胞现象。“组织细胞”存在于扩张淋巴管的管腔内。病变周边有淋巴样聚集物和被浆细胞包绕的厚壁血管。用抗S - 100蛋白抗体染色显示，每例“组织细胞”的胞质均有明显阳性。两组之间唯一不同的特征是，在一例仅限于皮肤的病例中存在假上皮瘤样增生和嗜酸性粒细胞浸润。我们认为皮肤窦组织细胞增生症可通过皮肤活检进行特异性诊断。由于皮肤窦组织细胞增生症可能不为普通病理学家、皮肤病理学家和皮肤科医生所熟知，仅限于皮肤的病例可能未得到充分认识。
