朗格汉斯细胞组织细胞增生症伴发急性呼吸窘迫综合征1例

A Case Report of Letterer-Siwe Disease with ARDS

患儿男,6个月。头、面、颈、躯干及双手(足)相继出现红色丘疹及结痂3个月,伴发热、咳嗽、腹泻、肝脾、淋巴结肿大及贫血。皮损组织病理示:真皮浅层致密组织样细胞浸润,浸润细胞的胞质丰富,部分细胞核可见核沟,表皮内散在分布类似细胞。免疫组化示:浸润细胞S100和CD1a抗原强表达。诊断:朗格汉斯细胞组织细胞增生症。予强的松、长春新碱及依托泊苷联合化疗后病情缓解,但患儿最后发展为急性呼吸窘迫综合症而死亡(ARDS)。

A 6-month-old boy presented with reddish papules and crusts on scalp, face, neck, trunk, hands and feet for 3 months ,accompanied by fever, cough ,diarrhea, hepatosplenomegaly, lymphadenectasis and anemia. The skin biopsy revealed dense infiltration of histiocytes like cells with abundant eosinophilic cytoplasm in the superficial layer of the dermis and disperse distribution in the epidermis. Immunohistochemical stains in- dicated the cells strong positive for S100 and CD1 a. The diagnosis of Letterer-Siwe disease （Langerhans cell histiocytosis） was made. The patient was in remission after combination chemotherapy of predinisone, vin- cristine sulfate and etoposide,but finally he died of acute respiratory distress syndrome.