先天性自愈性朗格汉斯组织细胞增生症

Congenital self healing Langerhans cell histiocytosis

报告1例先天性自愈性朗格汉斯组织细胞增生症。患儿男,2个月。出生时即有全身多发性皮肤结节,皮肤科检查:左耳后、左面部、胸部、背部、左下肢、右示指共6处鲜红色结节,中央形成火山口状溃疡,组织病理示结节由梭形细胞构成,部分细胞胞质丰富,嗜酸性,可见多角、多核瘤巨细胞,间质纤维丰富,局部瘤细胞增生活跃。免疫组化:CD1a(+),S-100蛋白(±),CD68(-),切除5处皮肤结节,右示指处结节未切除,在10个月内自行消失,结合患儿临床症状及随访结果诊断为先天性自愈性朗格汉斯组织细胞增生症。

A 2-month-old male was born with 6 nodules on the left posterior auris, left face, the breast, the back ,the left leg and right index finger. Physical examination showed red nodules with central erateriform ulceration. Histopathological examination revealed spindle cells, some were mononuelear histiocytes with abundant eosinophilic or foamy cytoplasm.Polynucleart tumor giant cells and abundant interstitial substance were seen in the dermis. These mononuelear histiocytoid cells were stained positive for S-100 protein and CDla negative for CD 68. 5 nudules were excised, and spontaneous resolving occurred to the remaining nodule in the right index finger within 10 months. The diagnosis of congenital self-healing Langerhans cell histiocytosis was finally established.