Pemphigoid gestationis: a rare case and review

Abstract

Pemphigoid gestationis is a rare, autoimmune blistering dermatosis (Holmes and Black In: Clin Exp Dermatol 7:65-73, 1982). It is unique in that it is diagnosed primarily in association with pregnancy. It has been estimated to occur in 1:50,000 pregnancies [Shornick et al. In: J Am Acad Dermatol 8:214-224, 1983]. Involvement of the infant is exceedingly rare, while the pathogenic trigger initiating disease has remained elusive; the interaction of IgG1 auto-antibodies and the 180 kD BPA2 remains paramount in understanding the immunologic mechanism of disease. We describe a 29 year-old multiparous woman who presented at 29 weeks gestation with skin findings characteristic of pemphigoid gestationis. She later gave birth to a full-term male who was born with extensive bullae and several denuded areas of his head, trunk and limbs. The diagnosis of pemphigoid gestationis in both mother and son was confirmed using direct immunofluorescence. In conclusion, we present a well documented, rare case of pemphigoid gestationis with maternal transfer to the infant. A historical and epidemiological review of this rare blistering disorder is discussed along with current theories on pathogenic and immunologic mechanisms.