红皮病性大疱性类天疱疮1例  

Erythrodermic bullous pemphigoid: a case report

作　　者：张亚利 李小红[1] 杨秋萍 张曼曼 张江安[1] 于建斌[1] ZHANG Yali;LI Xiaohong;YANG Qiuping;Zhang Manman;ZHANG Jiang′an;YU Jianbin(Department of Dermatology,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院皮肤科,河南郑州450052

出　　处：《皮肤性病诊疗学杂志》2021年第6期476-479,共4页Journal of Diagnosis and Therapy on Dermato-venereology

基　　金：河南省高等学校重点科研项目(20A320072)。

摘　　要：患者女,78岁。因"躯干、四肢红斑、鳞屑伴瘙痒5年,加重3年余"入院,入院后1周大腿及腹部出现紧张性水疱。住院第6天、第9天血清抗BP180抗体均阳性,IgE>2 500 U/mL。皮损组织病理可见表皮下水疱,真皮浅层大量淋巴细胞为主的浸润。直接免疫荧光示:基底膜带IgG和C3线状沉积,IgA阴性。诊断:红皮病性大疱性类天疱疮。给予甲泼尼龙40 mg静脉滴注1天1次,治疗1周,病情好转出院。1年后随访,患者泼尼松片减量至15 mg口服1天1次,躯干四肢可见轻度红斑,无水疱、糜烂和瘙痒。A 78-year-old female presented with scattered erythema and scale with itching on the trunk and limbs for 5 years and aggravated for more than 3 years. There were scattered tension blisters on the thigh and abdomen after one week during inpatient treatment. On the 6 th and 9 th day of hospitalization, the serum anti-BP180 antibody was positive, IgE>2 500 U/mL. Histopathology showed subepidermal blister and dense lymphocytes infiltration of the upper dermis. Direct immunofluorescence of perilesional skin demonstrated linear deposition of IgG and C3 along the basement membrane zone(BMZ). The diagnosis of erythrodermic bullous pemphigoid was made. After one week of treatment with prednisone(40 mg daily), these symptoms of blisters and erythroderma were substantially improved. One year after treatment, the dosage of prednisone was gradually reduced to 15 mg daily. Mild erythema only could be seen on the trunk and limbs, without blisters, erosion and itching.

关 键 词：大疱性类天疱疮 红皮病 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]