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.

Descriptor ID	D016506
MeSH Number(s)	C16.320.850.700 C17.800.827.700 C17.800.865.858
Concept/Terms	Pemphigus, Benign Familial Pemphigus, Benign Familial Benign Familial Pemphigus Familial Pemphigus, Benign Benign Chronic Pemphigus Familial Benign Chronic Pemphigus Hailey-Hailey Disease Hailey Hailey Disease

Below are MeSH descriptors whose meaning is more general than "Pemphigus, Benign Familial".

Below are MeSH descriptors whose meaning is related to "Pemphigus, Benign Familial".

Below are MeSH descriptors whose meaning is more specific than "Pemphigus, Benign Familial".