An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and 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 - both have abnormal, unstable between and defective . It is unrelated to though it closely resembles that disease.
Maladie de Hugh-Hailey /
Pemphigus bénin chronique familial /
Dermatose acantholytique familiale /
Maladie de Hailey et Hailey /