发疹性朗格汉斯细胞组织细胞增生症1例

A case report of exanthematous Langerhans cell histiocytosis

报告1例发疹性朗格汉斯细胞组织细胞增生症。患者男,27岁。四肢、躯干皮肤粟粒至黄豆大播散性丘疹、结节4年余,不伴系统损害,进展缓慢。皮损组织病理检查示真皮团块状组织细胞浸润,免疫组化染色结果示S-100蛋白(+),CD1a(+),LYSO(+),电镜下组织细胞内找到Birbeck颗粒。经糖皮质激素软膏外用,皮损有所消退,但停药后复发。随访2年,患者皮损进展缓慢,其他系统未累及。

A 27-year-old male patient with exanthematous Langerhans cell histiocytosis is reported. Clinical manifestation of this case was characterized as progressing slowly exanthematous papules and nodules sized from foxtail millet to soybean, without systemic damage. Immunohistochemical staining showed that the large histocytic cells infiltrated in the dermis were positive for S-100, CDla and LYSO. Birbeck granules were also found in those cells under the electron microscopy. Skin lesion was improved after the topical application of glucocorticoid. However, the recurrence was observed after the drug withdrawal. During 2 year follow-up, the skin lesion progressed slowly with no other systemic involvement.