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Hailey-Hailey Disease: Case Series and Review of Systemic Medications Publisher



Balighi K1 ; Razavi Z1 ; Daneshpazhooh M1 ; Lajevardi V1 ; Kamyabhesari K1 ; Ghafouri K2
Authors
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Authors Affiliations
  1. 1. Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Dermatology Practical and Conceptual Published:2024


Abstract

Introduction: Hailey-Hailey disease (HHD) is a rare inherited blistering skin disorder characterized by a chronic relapsing course.While it does not pose a serious threat to the patient's health, the quality of life can change. Unfortunately, there is currently no standard treatment for this condition. Objectives: In this observational retrospective cohort study, our aim was to discover the demographic characteristics and treatment strategies for managing HHD. Methods: In this retrospective cohort study, we documented the demographic, clinical, and histo-pathological characteristics beside various treatment employed options of patients diagnosed with HHD at Razi Hospital over the past 14 years. Results:A total of 32 patients with HHD were enrolled in the study (15 male and 17 female).The meanage of patients was 50.41 ± 13.15 (22-77) years.The average age of disease onset was 37.31 ± 11.88(15-60) years.Among the participants, 16 individuals (50%) affirm a positive family history of somekind of pemphigoid blisters.The most common site of disease activity was the inguinal area, observed in14 patients (33.33%). Histopathological examination discovered the existence of suprabasal acanthol-ysis in all of the specimens.Worthily,direct immunofluorescence analysis showed negative results in allskin biopsies.All patients received topical steroids and either topical or systemic antimicrobial agents.In cases of flares, systemic steroids were the most popular and favorable treatment choice during flares. Conclusion: Indeed, Hailey-Hailey disease, characterized by its chronic inflammatory and rare nature witha relapsing and remitting course, poses a significant challenge for dermatologists.The treatment of HHDhas been less than satisfactory and it often presents a challenge and could be misdiagnosed.Among theavailable treatment options, topical steroids and antimicrobial agents are the most administered therapies. © 2024 Balighi et al.