先天性自愈性朗格汉斯组织细胞增生症1例

A Case of late-onset Congenital Self-healing Langerhans Cell Histiocytosis

患儿男,10个月,全身多发结节1个月。皮肤科情况:头皮、额头、臀部、双下肢、双足、阴囊散在分布红褐色结节,黄豆至豌豆大小,表面无破溃。皮损组织病理示:真皮浅层可见灶状或片状分布的组织细胞样细胞,胞质丰富,淡嗜伊红染,核椭圆形,有核沟,核分裂偶见。并见散在淋巴细胞、中性粒细胞浸润。免疫组织化学:CD1a(+),S-100(+)。皮疹在3个月内自行消退。诊断:先天性自愈性朗格汉斯组织细胞增生症。

A 10-month-old boy presented with skin rashes for a mouth. Dermatological examination showed soybean to pea sized nodules without rupture on his scalp, forehead, hips,legs, feet and scrotum. I-Iistopathological examination revealed localized infiltrate of histiocyte - like cells with abundant eosinophilic cytoplasm, oval nuclei with nuclear grooves and a few mitotic figures. Sporadic lymphocytes and neutrophils could be seen in the superficial dermis. These histiocyte - like cells were positive for S-100 protein and CDla. The skin rashes resolved spontaneously within 3 months. The diagnosis of congenital self - healing Langerhans cell histiocytosis was finally established.