Department of Dermatology, the Second Xiangya Hospital, Central South University, Changsha, China.
Am J Dermatopathol. 2021 Jan 1;43(1):e9-e12. doi: 10.1097/DAD.0000000000001724.
Rosai-Dorfman disease (RDD, also known as sinus histiocytosis with massive lymphadenopathy) is a rare and benign non-Langerhans cell histiocytosis. Skin biopsy usually shows nodular or diffuse dermatitis. Rosai-Dorfman cells (RDD cells) and emperipolesis are the key to diagnosis. RDD cells express S-100 antigen, CD68, CD163, α1-antitrypsin, α1-antichymotrypsin, and ham-56, whereas Langerhans cell markers such as CD1a and langerin are negative. We presented a case of a 55-year-old man with varying sizes of many dark red nodules and lumps over the face, trunk, and limbs for approximately 1 year but without systemic involvement. The results of the laboratory evaluations were notable for an increased level of serum IL-6 and serum IgG4. Histopathological examination showed a diffused dense nodular infiltration of "nude" epithelioid histiocytes with infiltration of minimal lymphocytes and plasm cells around the epithelioid nodules. Immunohistochemistry identified nodular histiocytes being stained strongly positive for S-100 and CD68 but negative for CD1a. Plasma cells showed focally positive for IgG, IgG4, and CD38 and with a ratio of IgG4/IgG >40%. Considering these findings, we believe that our case meets the diagnostic description of "cutaneous Rosai-Dorfman disease" and is, therefore, a rare case with clinical features of multiple tumor-like nodules, sarcoidosis-like histological features, and immunohistochemistry of IgG4-positive plasma cells.
罗道氏病（RDD，也称为伴有巨大淋巴结病的窦组织细胞增生症）是一种罕见的良性非朗格汉斯细胞组织细胞增生症。皮肤活检通常表现为结节性或弥漫性皮炎。罗道氏细胞（RDD 细胞）和吞噬现象是诊断的关键。RDD 细胞表达 S-100 抗原、CD68、CD163、α1-抗胰蛋白酶、α1-抗糜蛋白酶和 ham-56，而朗格汉斯细胞标志物如 CD1a 和 langerin 则为阴性。我们报告了一例 55 岁男性患者，他面部、躯干和四肢有大小不一的许多暗红色结节和肿块，大约 1 年没有全身受累。实验室检查结果显示血清 IL-6 和血清 IgG4 水平升高。组织病理学检查显示“裸”上皮样组织细胞弥漫性致密结节性浸润，上皮样结节周围浸润有少量淋巴细胞和浆细胞。免疫组织化学鉴定结节状组织细胞对 S-100 和 CD68 染色呈强阳性，但对 CD1a 染色呈阴性。浆细胞对 IgG、IgG4 和 CD38 呈局灶性阳性，且 IgG4/IgG>40%。考虑到这些发现，我们认为我们的病例符合“皮肤罗道氏病”的诊断描述，因此是一种罕见的病例，具有多瘤样结节的临床特征、类肉瘤样组织学特征和 IgG4 阳性浆细胞的免疫组织化学特征。
