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Genetically Confirmed Localized Perianal Hailey-Hailey Disease Successfully Treated With Topical Difamilast: A Case
Maho Maekawa1, Kenji Yoshida1, Keiji Tanese1
1First Department of Dermatology, Toho University School of Medicine, Tokyo, JPN.
Cureus
|May 20, 2026
Summary
Hailey-Hailey disease, a rare skin condition, can present atypically in the perianal region. Topical difamilast showed promise in treating this localized form, improving symptoms and healing lesions.
Area of Science:
- Dermatology
- Genetics
- Pathology
Background:
- Hailey-Hailey disease (familial benign chronic pemphigus) is a rare autosomal dominant disorder.
- Typically affects intertriginous areas, but perianal localization is exceptionally rare.
- Characterized by genetic mutations in ATP2C1, leading to acantholytic dermatosis.
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