Pemphigoid Gestationis: Clinical and Laboratory Evaluation

Abstract

INTRODUCTIONPemphigoid 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.METHODSWe 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.DISCUSSIONDemographic 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 C3 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.CONCLUSIONThis 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.

Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis

“…Diverse immunosuppressants, including methotrexate, azathioprine, gold, pyridoxine, sulfapyridine, dapsone, and rituximab, can be considered postpartum for treatment of recalcitrant disease. 1,2,4,[38][39][40][41] The combination of a tetraycline with nicotinamide can also be an effective steroid-sparing option in non-lactating mothers. 42,…”

Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine

“…However, its use in pregnancy is limited by the risk of a B-lymphocyte depletion in the foetus. The use of azathioprine has been reported in previous cases 7,11 Again, the use of azathioprine during pregnancy for PG is difficult to justify because of risk of foetal marrow suppression and subsequent immunosuppression. Breastfeeding is not recommended while being on any of the systemic agents mentioned above.…”

Pemphigoide Gestationis a Propos Dun Cas Et Revue De La Litterature.

“…La PG se manifeste cliniquement par une éruption prurigineuse, érythémato-papuleuse, urticarienne, parfois en cocarde, plus ou moins étendue, débutant classiquement sur le ventre mais pas toujours, évoluant vers l'apparition inconstante (60 à 80 % des cas) mais évocatrice de vésicules et bulles. Sont surtout touchés le tronc (notamment la région péri-ombilicale) et les membres inférieurs; l'atteinte du visage est possible ainsi que celle de la muqueuse buccale, mais plus rarement (0 à 15-20 % selon les études [5,6,7]). Le principal diagnostic différentiel est la dermatose polymorphe de la grossesse, se présentant sous la forme d'une éruption eczématiforme ou urticarienne prurigineuse habituellement non bulleuse [5,8].…”

“…Les quatre autres patientes ont été traitées avec succès par dermocorticoïdes et la durée moyenne du traitement a été dans tous les cas (dermocorticoïdes et corticothérapie générale) de 12 semaines [16]. L'expérience d'autres équipes reste pourtant plutôt en faveur de la corticothérapie générale, comme celle de Cobo et al qui rapportent sept patientes, dont six ont eu recours à une corticothérapie orale à forte dose, de 0,5 à 1 mg/kg/j, a priori en première intention [7]. Aucun effet indésirable maternel ou foetal spécifique de la corticothérapie locale ou générale n'est rapporté dans ces études.…”