角层下脓疱病误诊为慢性家族性良性天疱疮1例  

A Case of Subcorneal Pustular Dermatosis Misdiagnosed as Familial Benign Chronic Pemphigus

作　　者：赵妍[1] 杨旭芳 邹先彪[1] ZHAO Yan;YANG Xufang;ZOU Xianbiao(The Fourth Medical Center of PLA General Hospital,Beijing 100048,China)

机构地区：[1]解放军总医院第四医学中心,北京100048

出　　处：《中国皮肤性病学杂志》2021年第5期586-589,共4页The Chinese Journal of Dermatovenereology

摘　　要：患者女,16岁,褶皱处反复脓疱、糜烂16年,双大腿红斑脓疱伴瘙痒1周。患者既往曾诊断为"慢性家族性良性天疱疮"。右大腿皮损组织病理:角质层下可见脓疱形成,棘层上方或角层下中性粒细胞浸润,真皮层小血管周围水肿,淋巴细胞灶性浸润,符合脓疱病。右侧腋下皮损组织病理:表皮角质层下见大疱形成伴中性粒细胞浸润,表皮脚向下延伸呈网状,真皮层血管周围见淋巴细胞浸润。免疫荧光标记:IgG、IgA、IgM、C3均为阴性。根据该患者临床表现,易误诊为慢性家族性良性天疱疮,结合病理及免疫荧光,最终诊断为角层下脓疱病。A 16-year-old female patient presented with recurrent pustules and erosions on the folds for 16 years,who had been suffering from erythema and pustules on both thighs accompanied with itching for 1 week.She had been initially diagnosed as familial benign chronic pemphigus.The histopathology of the lesion on her right thigh showed pustules below the stratum corneum,neutrophil infiltration above or below the stratum spinosum,small perivascular edema of the dermis,and focal infiltration of lymphocytes,which indicated pustulosis.The histopathology of the lesion on the right axilla showed bullae below the epidermal stratum corneum with neutrophil infiltration,the reticular extension of epidermal foot,and lymphocytes infiltration around the dermis.The immunohistochemistry tests showed the IgG,IgA,IgM and C3 were negative.The patient was initially misdiagnosed as having familial benign chronic pemphigus due to clinical manifestations.Combined with histological and immunofluorescence tests,she was finally diagnosed as subcorneal pustular dermatosis.

关 键 词：角层下脓疱病 天疱疮 慢性家族性良性 误诊 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]