Familial pemphigus vulgaris with oral manifestations affecting two Greek families.

Two families of Greek origin with oral pemphigus vulgaris are reported. Both are fully documented cases of familial pemphigus in Greek Mediterranean patients. In the first family, a sister and a brother developed oral mucosal pemphigus lesions within a 5-year period, and a cousin had died from the disease 20 years earlier. In the second family, a daughter, a son and their mother developed the disease 2, 4 and 6 years apart. HLA typing has been performed and disclosed striking associations and similarities between the members of each family. Review of the fully documented cases of familial pemphigus vulgaris is also presented in a tabular form.