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Late-onset Morbus Hailey-Hailey.

Eur J Dermatol

April 2024

Department of Dermatology, Maastricht University Medical Center+, Center of Expertise for Genodermatoses, Maastricht, The Netherlands, GROW Research Institute for Oncology and reproduction Maastricht University, Maastricht, The Netherlands, Dermadok Huidkliniek, Antwerp, Belgium.

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Hailey-Hailey disease (HHD) is a rare, late-onset autosomal dominant genodermatosis characterized by blisters, vesicular lesions, crusted erosions, and erythematous scaly plaques predominantly in intertriginous regions. HHD is caused by ATP2C1 mutations. About 180 distinct mutations have been identified so far; however, data of only few cases from Central Europe are available.

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Identification of subtle disease-specific histologic changes may be of significant help in early diagnosis of acantholytic skin diseases. Hailey-Hailey disease (HHD) is an autosomal dominant genodermatosis characterized by vesiculoerosive lesions favoring the intertriginous areas. Histologically, HHD is characterized by full-thickness acantholysis of the spinous layer in association with dyskeratosis of individual keratinocytes; a pemphigus vulgaris-like suprabasal pattern of acantholysis may be observed in the earliest stages of disease.

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Hailey-Hailey disease: A fold (intertriginous) dermatosis.

Clin Dermatol

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Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul 34098, Turkey.

Hailey-Hailey disease, also called benign familial pemphigus, is a late-onset blistering disorder that affects the flexures. There are typically painful erosions and cracks in affected areas. Lesions generally begin between 20 and 40 years of age.

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