Pemphigus vulgaris (PV) is a rare, blistering autoimmune disease that affects the skin and mucous membranes. Patients have circulating antibody to an intercellular cement substance, and deposition in vivo of this antibody is a hallmark of the disease. ... Pemphigus vulgaris (PV) is a rare, blistering autoimmune disease that affects the skin and mucous membranes. Patients have circulating antibody to an intercellular cement substance, and deposition in vivo of this antibody is a hallmark of the disease. The antibody appears to be pathogenetic, since newborn infants of mothers with pemphigus may have blisters, and newborn mice injected with the antibody from patients have clinical pemphigus. The disease is reported to have a particularly high incidence among Jews (summary by Ahmed et al., 1990).
Pemphigus vulgaris has rarely been observed in multiple members of families, and in many of the reported cases, full histopathologic and/or immunopathologic confirmation has been lacking. The first documented report of familial occurrence was that of Voelter et ... Pemphigus vulgaris has rarely been observed in multiple members of families, and in many of the reported cases, full histopathologic and/or immunopathologic confirmation has been lacking. The first documented report of familial occurrence was that of Voelter et al. (1973). Laskaris et al. (1989) described 2 Greek families in which multiple members were affected and manifestations were limited largely to the oral cavity. Alijotas et al. (1990) reported a 10-year-old boy and his 8-year-old sister who achieved prolonged remission after cyclosporine therapy.