CAJ | 학술논문

患者男,34岁.因双侧腋部皮肤溃疡久不愈合,伴疼痛1年,胸闷、乏力、多尿3年就诊.具有尿崩症、突眼、皮肤黏膜病变(包括浸润结节和溃疡斑块,特别是在腋下,生殖器区域).其他尚见广泛的皮疹、脱屑或结痂性丘疹.组织学特点为真皮全层弥漫分布的朗格汉斯组织细胞.瘤细胞表达CD1a、S-100.组织病理及免疫组化检查符合朗格汉斯组织细胞增生症(Hand-Schuller-Christian病).本病目前尚无特异性的治疗方法.多系统病变采取化疗,单发病灶者手术及放疗疗效好.
환자남,34세.인쌍측액부피부궤양구불유합,반동통1년,흉민、핍력、다뇨3년취진.구유뇨붕증、돌안、피부점막병변(포괄침윤결절화궤양반괴,특별시재액하,생식기구역).기타상견엄범적피진、탈설혹결가성구진.조직학특점위진피전층미만분포적랑격한사조직세포.류세포표체CD1a、S-100.조직병리급면역조화검사부합랑격한사조직세포증생증(Hand-Schuller-Christian병).본병목전상무특이성적치료방법.다계통병변채취화료,단발병조자수술급방료료효호.
[Objective] A case of 34-year-old male was admitted for persistant painful skin ulcer in both axillary regions for 1 year as well as oppression in the chest,fatigue and polyuria for 3 years.He also suffered from diabetes insipidus and exophthalmos.Skin examination showed infiltrated nodules and ulcerated plaques mainly located in the subaxillary and genital regions,as well as widespread skin rashes,scaly or crusted papules.Histopathology revealed a diffuse infiltrate of Langerhans's cells throughout the entire dermis.The tumor cells were positive for CD1a and S-100.Finally,a diagnosis of Hand-Schüller-Christian disease was made based on the histopathological and immunohistochemical findings.At present,no specific treatment is available for Hand-Schüller-Christian disease.Generally speaking,combined chemotherapy is recommended for Hand-Schüller-Christian disease with multisystemic involvement,and surgical operation or radiotherapy for unifocal lesions.