Hailey-Hailey disease: A review of clinical features in 26 cases with special reference to the secondary infections and their control

Background/ObjectiveHailey-Hailey disease (HHD) is a rare autosomal dominant hereditary disorder that manifests with skin blisters. Controlling secondary infections is indispensable for this condition in conjunction with treatment using anti-inflammatory drugs such as topical steroids. This study aimed to investigate the clinical features and secondary infections associated with HHD.MethodsWe retrospectively reviewed the records of 26 patients affected by HHD who visited our hospital over a 10-year period. We explored the patients' age at onset, time from onset to diagnosis, affected sites, symptoms, secondary infections and their control, other treatment responses, and family history for HHD.ResultsThe mean age at disease onset was 35 years. The average time from onset to diagnosis was 7 years. The lesions were located at the neck, inframammary folds, axillae, groin, and perineum. Secondary infection accompanied HHD in 22 patients (85%); the most common infectious agents were Staphylococcus aureus and Candida albicans. Our cases demonstrated that first-line treatment for HHD was successful in most patients, which included topical corticosteroids in combination with topical or oral antibiotics and/or antifungal agents.ConclusionControlling the secondary infection is indispensable for treatment of skin lesions in HHD; however, none of the treatment modalities prevented relapse after discontinuation of treatment.