表现为播散性丘疹型的皮肤型窦组织细胞增生症一例

A case of disseminated papular cutaneous Rosai-Dorfman disease

患者男,54岁,全身红色丘疹、结节及斑块4个月。皮损初发于双下肢,渐波散至全身。查体示全身密集分布的红色丘疹、结节,孤立或呈环形、条带状分布,直径0.3~2.5 cm,质地较硬,略有压痛,有浸润感;皮损大致对称分布,以四肢伸侧、面颊部为著。组织病理示真皮内弥漫性组织细胞浸润,多个具有分枝的肥大的组织细胞及多核巨细胞浸润,灶性浆细胞、淋巴细胞、中性粒细胞浸润,少许嗜酸粒细胞浸润,有细胞伸入现象。免疫组化检查示肥大的组织细胞及多核巨细胞CD68、CD163、S-100蛋白均阳性,CD1a阴性。诊断:皮肤型窦组织细胞增多症。给予复方甘草酸苷口服及卡介菌多糖核酸肌内注射1个月,皮损部分消退。

A 54-year-old male patient presented with multiple red papules, nodules and plaques over the whole body for 4 months. The lesions occurred firstly in the lower limbs and gradually scattered to the whole body. Dermatological examination showed dense deep red papules and nodules arranging in solitary, circular, or strip shape line, about 0.3~2.5cm in diameter, with a hard texture, slight tenderness, and the base of lesions were slightly infiltrative. The lesions were more severe on the extensor of the extremities and cheek, and roughly symmetric. Pathological examination showed diffuse histiocytic infiltration in the dermis, with multiple branched hypertrophic histiocytes and multinucleated giant cells, and focal infiltrate of plasma cells, lymphocytes, neutrophils and a little of eosinophils, with the phenomenon of emperipolesis. Immunohistochemical examination showed that hypertrophic cells and multinucleated giant cells were stained positive for CD68, CD163, S100, and negative for CD1 a. The diagnosis was cutaneous sinus histiocytosis. The patient was treated with oral compound glycyrrhizin tablets and intramuscular injection of BCG polysaccharide and nucleic acid injection for 1 month, and the lesions subsided partly.