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Familial benign pemphigus ��HHD�� is a rare autosomal dominant inherited disorder characterized by recurrent multiple vesicles and bullae predominantly localized in body folds. In 2000, it was confirmed that HHD is caused by a heterozygous mutation in the ATP2C1 gene, which encodes the human secretory pathway Ca2+/ Mn2+-ATPase protein 1 ��hspcA1�� . At present, more than 100 kinds of mutation points have been detected. This paper offers an update on the knowledge about the etiology, pathogenesis, clinical manifestations, histopathology, treatment of HHD, so as to provide a theoretic basis for the research of HHD.