Pemphigoid gestationis: clinical and laboratory evaluation

Abstract

Introduction: Pemphigoid gestationis, also known as herpes gestationis, is a rare autoimmune blistering disease associated with pregnancy. It usually occurs during the second or third trimester, but it may be present at any stage of pregnancy or the puerperium. The clinical, histologic, and immunopathological features of pemphigoid gestationis are similar to those of the pemphigoid group of disorders.

Methods: We hereby report seven patients who were diagnosed with pemphigoid gestationis and followed at the Autoimmune Blistering Disease Clinic in the Department of Dermatology of the University of Sao Paulo Medical School between 1996 and 2008.

Discussion: Demographic and clinical findings, such as median age, sites of involvement, and gestational age of onset or C3 of our patients, coincide with those described in previous reports. The majority of patients (85%) exhibited complement C(3) and immunoglobulin G (IgG) deposition along the basement membrane zone (BMZ) on immunofluorescence. Herpes gestationis factor (HG) factor was postitive in four out of six patients (67%), and three out of five patients recognized the bullous pemphigoid recombinant antigen (BP180) by ELISA.

Conclusion: This study revealed a good outcome of the newborns from pemphigoid gestationis affected mothers, based on the absence of pemphigoid gestationis cutaneous lesions, mean birth weight, and normal Apgar scores and gestational age at birth.

Keywords: Autoimmunity; BP180; Blistering disease; Dermatoses of pregnancy; Herpes gestationis.

Figure 1

Pemphigoid Gestationis: Pruritic urticarial lesions and tense blisters on the trunk

Figure 2

Pemphigoid Gestationis: Histopathology (HE): subepidermal bulla with eosinophils (X40)

Figure 3

Pemphigoid Gestationis: Direct immunofluorescence of peri-lesional skin, showing linear deposition of complement (C3) along the BMZ (X40)