Pemphigus, Benign Familial


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.