The presence of circulating pemphigus vulgaris antibodies (PV-IgG) in first degree releatives of Jewish pemphigus patients

The distribution of pemphigus vulgaris-IgG subclasses and their reactivity with desmoglein 3 and 1 in pemphigus patients and their first-degree relatives

“…The results of our study confirm previously reported findings that about 50% of healthy unaffected family members of PV patients do have circulating PV‐IgG. 12–14 The rarity of familial pemphigus, however, raises the question as to which factor(s) prevent PV‐IgG carriers among family members from developing clinical symptoms of PV and whether these factors might be linked to PV‐IgG subclasses.…”

“… 8 DNA analysis has revealed linkage between the alleles DRB*0402 and DRB*1401 and the presence of PV antibodies in patients and their first‐degree relatives. 9–11 Between 40% and 71% of immediate relatives of pemphigus patients have been found to be carriers of low‐titre PV autoantibodies, 12–14 findings that some consider to suggest a dominant inheritance. 10 A frequent occurrence of familial pemphigus could thus be expected, but this is not the case.…”