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.