NIH

Zebra of the Week: Hailey-Hailey disease

December 17, 2024

Hailey-Hailey disease, also known as familial benign chronic pemphigus, is a genetic skin disorder characterized by painful blistering and erosions, primarily in intertriginous areas such as the axillae, groin, neck, inframammary folds, and intergluteal cleft.

Symptoms often worsen with heat, sweating, and friction, leading to secondary bacterial infections. The condition is caused by mutations in the ATP2C1 gene and is inherited in an autosomal dominant manner. Diagnosis typically involves clinical evaluation and genetic testing. Management focuses on reducing symptoms and preventing infections.

Source:

NIH: Genetic and Rare Diseases Information Center. (Accessed 2024, December 16). Hailey-hailey disease. https://rarediseases.info.nih.gov/diseases/6559/hailey-hailey-disease