Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms
ABSTRACT Background Hailey‐Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis characterized by chronic, painful, erythematous, erosive plaques and fissures at sites of friction such as the neck, axilla, groin, and perineum. The pathogenesis is due...
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| Format: | Article |
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Wiley
2025-07-01
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| Series: | Health Science Reports |
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| Online Access: | https://doi.org/10.1002/hsr2.71061 |
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| author | Mahesh Mathur Sumit Paudel Nabita Bhattarai Sambidha Karki Sandhya Regmi |
| author_facet | Mahesh Mathur Sumit Paudel Nabita Bhattarai Sambidha Karki Sandhya Regmi |
| author_sort | Mahesh Mathur |
| collection | DOAJ |
| description | ABSTRACT Background Hailey‐Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis characterized by chronic, painful, erythematous, erosive plaques and fissures at sites of friction such as the neck, axilla, groin, and perineum. The pathogenesis is due to a mutation in the ATP2C1 gene, which encodes the human secretory‐pathway calcium/manganese‐ATPase isoform 1 (hSPCA1) that regulates calcium and manganese concentration in the Golgi apparatus. The diagnosis relies on clinical presentation and characteristic histopathological features, notably a “dilapidated brick wall” appearance. There is no cure for this genodermatosis and main aim of management in HHD is to control symptoms and reduce recurrence. Multitude of topical, systemic agents, procedural therapy such as laser therapy and surgery for the treatment of HHD have been reported in the literature. Aims This comprehensive review aims to discuss the efficacy of current HHD treatments with special focus on therapeutic mechanisms. Conclusion This review highlights clinical and histological features of HHD and offers guidance for dermatologists involved in managing this distinct dermatosis. |
| format | Article |
| id | doaj-art-0b2558f951734cf6baf6a5e24df5cb16 |
| institution | Kabale University |
| issn | 2398-8835 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Health Science Reports |
| spelling | doaj-art-0b2558f951734cf6baf6a5e24df5cb162025-08-20T03:44:46ZengWileyHealth Science Reports2398-88352025-07-0187n/an/a10.1002/hsr2.71061Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic MechanismsMahesh Mathur0Sumit Paudel1Nabita Bhattarai2Sambidha Karki3Sandhya Regmi4Department of Dermatology College of Medical Sciences Teaching Hospital Bharatpur NepalDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur NepalDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur NepalDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur NepalDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur NepalABSTRACT Background Hailey‐Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis characterized by chronic, painful, erythematous, erosive plaques and fissures at sites of friction such as the neck, axilla, groin, and perineum. The pathogenesis is due to a mutation in the ATP2C1 gene, which encodes the human secretory‐pathway calcium/manganese‐ATPase isoform 1 (hSPCA1) that regulates calcium and manganese concentration in the Golgi apparatus. The diagnosis relies on clinical presentation and characteristic histopathological features, notably a “dilapidated brick wall” appearance. There is no cure for this genodermatosis and main aim of management in HHD is to control symptoms and reduce recurrence. Multitude of topical, systemic agents, procedural therapy such as laser therapy and surgery for the treatment of HHD have been reported in the literature. Aims This comprehensive review aims to discuss the efficacy of current HHD treatments with special focus on therapeutic mechanisms. Conclusion This review highlights clinical and histological features of HHD and offers guidance for dermatologists involved in managing this distinct dermatosis.https://doi.org/10.1002/hsr2.71061ATP2C1 mutationfamilial benign chronic pemphigusgeneral dermatologygenodermatosisHailey‐Hailey disease |
| spellingShingle | Mahesh Mathur Sumit Paudel Nabita Bhattarai Sambidha Karki Sandhya Regmi Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms Health Science Reports ATP2C1 mutation familial benign chronic pemphigus general dermatology genodermatosis Hailey‐Hailey disease |
| title | Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms |
| title_full | Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms |
| title_fullStr | Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms |
| title_full_unstemmed | Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms |
| title_short | Hailey‐Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms |
| title_sort | hailey hailey disease an updated review with a focus on therapeutic mechanisms |
| topic | ATP2C1 mutation familial benign chronic pemphigus general dermatology genodermatosis Hailey‐Hailey disease |
| url | https://doi.org/10.1002/hsr2.71061 |
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