Annular Hailey–Hailey disease

Ariane Fizazi, Olivier Dereure
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Abstract

Hailey–Hailey disease (HHD) is a genodermatosis that typically presents with crusted erosions located in skin folds and the perineum. We report two cases of a highly atypical form of HHD, characterised by inflammatory annular skin lesions on the trunk, isolated and sparing the folds in one patient. Diagnosis was confirmed by histology or genetic analysis. Clinicians should consider this exceptional subset of HHD when encountering annular lesions resistant to antifungals even when a relevant familial background is lacking.

Abstract Image

环形海利-海利病
海利-海利病(Hailey-Hailey disease,HHD)是一种遗传性皮肤病,典型表现为皮肤皱褶和会阴部结痂性糜烂。我们报告了两例高度不典型的海利-海利病病例,其特征是躯干上出现炎性环状皮损,其中一名患者的皮损孤立存在,褶皱部位没有皮损。诊断是通过组织学或基因分析确认的。临床医生在遇到对抗真菌药物耐药的环状皮损时,即使没有相关的家族背景,也应考虑到 HHD 的这一特殊亚型。
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