Pemphigoid gestationis

Abstract

Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of complement along the basement membrane zone of perilesional skin. The disorder is accompanied by severe pruritus and polymorphous bullous skin lesions. Clinical diagnosis is confirmed by histology and positive cutaneous immunofluorescence and immunoelectron microscopy tests (linear deposition of C3, with or without immunoglobulin G, along the basement membrane zone, within the lamina lucida, and localized to the proximal part of anchoring filaments of the epidermal fragment of salt-split skin). Enzyme-linked immunosorbent assay for pemphigoid gestationis antibody (BP180) is commercially available. If local treatment fails, systemic corticosteroid therapy should be administered. Oral corticosteroids are the therapeutic mainstay in pregnancy and postpartum. The prognosis is good for mother and child, except that there is a risk of preterm delivery and of moderate fetal growth restriction. Recurrence is possible during subsequent pregnancies. There is no significant maternal morbidity or mortality.

Pemphigoid gestationis: A Moroccan study

“…Only the blood eosinophilia is suggestive [7]. In our series one patient was discovered as having hypercalcimia secondary to primary hyperparathyroidism.…”

“…Pemphigoid gestationis (PG) is intensely pruritic and a rare autoimmune vesiculobullous dermatosis of pregnancy closely related to the pemphigoid group of blistering disorders, with an incidence of 1:50,000 to 60,000 pregnancies [7][8][9]. Synonyms for PG include herpes gestationis, dermatitis multiformis gestationis, and gestational pemphigoid.…”

“…The dermatosis was originally named "herpes gestationis" by Milton in 1872 [8]. On the basis of the morphologic herpetiform feature of the blisters; however, the name was revised to PG, because PG is not related to or associated with any active or prior herpes virus infection [7].…”

Bullous eruption on the back of both hands in a newborn

“…Our case report shows the rare case of a passive transplacental passage of maternal autoantibodies to the fetus resulting in postpartal blistering on the skin of the newborn. The etiology of pemphigoid gestationis is uncertain and in contrast to its obsolete naming Herpes gestationis there is no association with viral herpes infections [5]. Most of the patients develop autoantibodies against the hemidesmosomal structural proteins BP180 and in rare cases against BP230, similar to bullous pemphigoid.…”

Cyclosporine Treatment in Severe Gestational Pemphigoid

“…The diagnosis is based on a positive direct immunofluorescence (DIF) analysis of a perilesional skin biopsy (5), and elevated BP180 antibody levels parallel disease activity (6). PG typically occurs in the second and 3 rd trimester of pregnancy with pruritic urticarial papules and plaques followed by blistering (5,7,8). In addition to maternal morbidity, PG is associated with prematurity and foetal growth restriction (8)(9)(10).…”