朗格汉斯细胞组织细胞增生症并发多系统受累

Multisystem langerhans cell histocytosis: a case report

报道1例多系统受累的朗格汉斯细胞组织细胞增生症。患者男,20岁,甲变形、部分缺如8年余,皮损及甲状腺增大5年,肝功能异常1年,黄疸5个月,多饮多尿1个月。皮肤科检查:面、颈、躯干及四肢充血性、出血性浸润性暗红色丘疹、斑块,甲变形缺如。面部皮损组织病理检查:真皮内见大片浸润性分布的肿瘤细胞,细胞胞质丰富,红染,核大,呈不规则形,核分裂象可见;免疫组织化学染色:S-100蛋白,CD1a均阳性。诊断:朗格汉斯细胞组织细胞增生症。治疗:予化疗及对症治疗,疗效不佳。

A case of multisystem langerhans cell histocytosis(LCH) is reported. A 20-year-old man presented with deformed nails for more than 8 years, skin lesion and enlarged thyroid gland for 5 years, hepatic dysfunction for 1 year, jaundice for 5 months, polyuria and polydipsia for 1 month. Physical examination showed many erythematous or hemorrhagic, indurated dark red papules and plaques on the face, neck, trunk and limbs. Most of his nails were deformed. Histopathological examination revealed a diffuse dermal infiltrate of mononuclear cells with abundant eosinophilic cytoplasm and irregular nuclei. Mitosis was present. The immunohistochemical staining showed that the dermal infiltrate was positive for S-100 protein and CD1 a. The diagnosis of multisystem LCH was made. Chemotherapy was given, but the curative effect was unsatisfied.