Pemphigus, Benign Familial
Synonyms
Benign Chronic Pemphigus
Benign Familial Pemphigus
Familial Benign Chronic Pemphigus
Familial Pemphigus, Benign
Hailey Hailey Disease
Hailey-Hailey Disease
An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. Mutations in the ATP2C1 gene (encoding the secretory pathway Ca2++/Mn2++ ATPase 1 (SPCA1)) cause this disease. It is clinically and histologically similar to DARIER DISEASE - both have abnormal, unstable DESMOSOMES between KERATINOCYTES and defective CALCIUM-TRANSPORTING ATPASES. It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease.