Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid

Neonatal pemphigus is a rarely reported transitory autoimmune blistering disease caused by transfer of maternal IgG autoantibodies to desmoglein 3 to the neonate through the placenta when the mother is affected with pemphigus. It is clinically characterized by transient flaccid blisters and erosions on the skin and, rarely, the mucuous membranes. Neonatal pemphigus vulgaris has never been reported to persist beyond the neonatal period and progress to adult disease. Gestational pemphigoid is an uncommon, pregnancy-associated, autoimmune blistering disease. This disease typically flares with delivery and then spontaneously resolves within months without treatment. In 5% to 10% of cases, the antibodies responsible for gestational pemphigoid are transferred to the neonate through the placenta, causing transitory blistering in the neonate. While both gestational pemphigoid and pemphigus vulgaris can occur during pregnancy, these clinically, histologically, and serologically distinct diseases are not known to occur simultaneously in the same patient. We describe a case of a 36-year-old woman with clinical evidence of mucocutaneous pemphigus, but not gestational pemphigoid, who had serum antibodies to the antigens responsible for pemphigus as well as those responsible for gestational pemphigoid. This patient gave birth to a neonate with neonatal pemphigus but no evidence of neonatal gestational pemphigoid. (J Am Acad Dermatol 2009;60:1057-62.)