皮损形态类似于光泽苔藓的朗格汉斯细胞组织细胞增生症一例

Langerhans cell histiocytosis mimicking lichen nitidus:a case report

报道1例表现为光泽苔藓样皮损的朗格汉斯细胞组织细胞增生症。患儿,女,19个月。因前胸、后背多发针尖大丘疹伴多饮、多尿1个月余就诊。皮损临床表现为全身泛发性光泽苔藓样丘疹。垂体平扫+增强MRI提示:垂体后叶短T1信号消失。皮损组织病理显示表皮角化过度,真皮浅层密集组织细胞浸润,细胞核呈肾形,胞浆丰富。免疫组化:CD1a、S-100、CD68均阳性。结合临床、组织病理和免疫组化检查,诊断为朗格汉斯细胞组织细胞增生症。全身化疗后,皮损完全消退,目前仍在随访中。

A case of Langerhans cell histiocytosis mimicking lichen nitidus was reported. A 19-month-old girl presented with multiple pinpoint papules on her chest and back,with polyuria and polydipsia for one month. The clinical manifestation is generalized papular eruption mimicking lichen nitidus. The plain and enhanced MRI scanning of pituitary showed the disappear of high signal（ at T1WI） of the posterior lobe of pituitary gland. Histopathological examination revealed that hyperkeratosis and histiocytic infiltration in the upper dermis. The pathologic cell had a kidney-shaped nulclei with plenty cytoplasm. The immunohistochemistry showed histiocytes are positive for CD1a,S-100,CD68. The diagnosis of Langerhans cell histiocytosis was made. She was treated with systemic chemotherapy and the lesions resolved completely. Now she has been followed-up regularly.