A family in which two sisters are affected by pemphigus is described. Literature research has revealed the presence of 25 families, comprising 53 patients, in which pemphigus existed in more than one member. The relationships between those affected were mainly parent-child or sibling-sibling. Pemphigus was found to be associated with specific HLA antigens: A26, Bw38, DRw4, and additionally occurred with a higher frequency in certain ethnic groups. The variety of etiologic factors in the pathogenesis of pemphigus is discussed and the significance of a genetic role elucidated.

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