A Rare Case of Atypical Recalcitrant Hailey-Hailey Disease and a Literature Review  

作　　者：Udoka Ogbuneke Emmanuel Odega Yakub Wahab Ibrahim Mustapha Abubakar Udoka Ogbuneke;Emmanuel Odega;Yakub Wahab Ibrahim;Mustapha Abubakar(Internal Medicine Trainee Southend Hospital, Mid and South Essex NHS Foundation Trust, Southend on Sea, UK;Internal Medicine Trainee, Morriston Hospital, Swansea Bay University Health Board, Swansea, UK;Emergency Medicine, Southend Hospital, Mid and South Essex NHS Foundation Trust, Southend on Sea, UK)

机构地区：[1]Internal Medicine Trainee Southend Hospital, Mid and South Essex NHS Foundation Trust, Southend on Sea, UK [2]Internal Medicine Trainee, Morriston Hospital, Swansea Bay University Health Board, Swansea, UK [3]Emergency Medicine, Southend Hospital, Mid and South Essex NHS Foundation Trust, Southend on Sea, UK

出　　处：《Case Reports in Clinical Medicine》2022年第9期358-363,共6页临床医学病理报告（英文）

摘　　要：Hailey-Hailey disease also known as familial benign chronic pemphigus is a rare bullous genodermatosis that affects intertriginous area symmetrically. It presents with flaccid blisters, erosions and maceration resulting in increased morbidity, reduced quality of life for affected patients. It is rare in occurrence with an incidence of rate of 1 in 50,000. It is diagnosed with a combination of clinical and histopathological findings. While there is no known cure, its relapsing remitting course can be managed with medication. This case describes an unusual presentation of familial benign chronic pemphigus with a late age of onset of symptoms, atypical distribution and resistant to multiple therapies.Hailey-Hailey disease also known as familial benign chronic pemphigus is a rare bullous genodermatosis that affects intertriginous area symmetrically. It presents with flaccid blisters, erosions and maceration resulting in increased morbidity, reduced quality of life for affected patients. It is rare in occurrence with an incidence of rate of 1 in 50,000. It is diagnosed with a combination of clinical and histopathological findings. While there is no known cure, its relapsing remitting course can be managed with medication. This case describes an unusual presentation of familial benign chronic pemphigus with a late age of onset of symptoms, atypical distribution and resistant to multiple therapies.

关 键 词：Internal Medicine DERMATOLOGY Atypical Hailey-Hailey’s Disease Benign Chronic Pemphigus 

分 类 号：R73[医药卫生—肿瘤]