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一种表皮下大疱性疾病。

A subepidermal blistering disorder.

作者信息

Moolla Yusuf

机构信息

Department of Internal Medicine, Addington Hospital, Durban, South Africa.

出版信息

S Afr Med J. 2016 Feb 22;106(3):263. doi: 10.7196/SAMJ.2016.v106i3.10129.

Abstract

A young woman presented with generalised tense blisters. There had been no previous drug exposure and she had no clinical signs of autoimmune disease or evidence of sepsis. Given the abrupt onset and clinical scenario, a skin punch biopsy was performed and a tentative diagnosis of linear IgA bullous dermatosis (LABD) was made. Dapsone, an immunomodulatory sulfone that has been supported for use in LABD by case reports and clinical observation, was administered with an adjunctive oral corticosteroid to accelerate resolution. The biopsy findings were supportive of a subepidermal blistering disorder, such as linear IgA bullous dermatosis. After just 5 days of therapy, dramatic improvement was noted and she continued to improve. LABD is an uncommon condition, with incidence rates reported to be ranging from less than 0.5 to 2.3 cases per million individuals per year.

摘要

一名年轻女性出现全身性紧张性水疱。她之前未接触过药物,也没有自身免疫性疾病的临床体征或败血症的证据。鉴于起病突然及临床表现,进行了皮肤打孔活检,并初步诊断为线状IgA大疱性皮肤病(LABD)。氨苯砜是一种免疫调节性砜类药物,病例报告和临床观察均支持其用于LABD的治疗,同时给予辅助性口服皮质类固醇以加速病情缓解。活检结果支持表皮下大疱性疾病,如线状IgA大疱性皮肤病。治疗仅5天后,病情就有显著改善,且持续好转。LABD是一种罕见疾病,据报道发病率为每年每百万人口少于0.5至2.3例。

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A subepidermal blistering disorder.一种表皮下大疱性疾病。
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