Successfully Treated Case of Pemphigoid Gestationis with Post-Partum Intravenous and Oral Steroids

Excerpt

Pemphigoid gestationis (PG) is a rare autoimmune blistering disease of pregnancy. PG has an estimated prevalence of 1 case in 50,000–60,000 pregnancies. It is characterized by pruritic, urticarial plaques with the development of tense vesicles and bullae within the lesions. PG has been associated with premature delivery, small-for-gestational-age infants. Recurrences with subsequent pregnancies are often more severe. Oral glucocorticoids are the mainstay of therapy. Differentiation of PG from pruritic urticarial papules and plaques of pregnancy is essential because management and outcomes differ. In instances in which clinical diagnosis is difficult, direct immunofluorescence tests, immunoblots, or ELISA studies of anti-basement membrane zone antibodies are useful in establishing the diagnosis. Signs and symptoms of the disorder typically present during the second or third trimester of pregnancy, but may occur at any time during the pregnancy or anywhere up to six weeks after giving birth. Most patients develop antibodies against 2 hemidesmosomal proteins, BP180 (BPAG2, collagen XVII) and less frequently BP230 [1]. The trigger for the development of autoantibodies in persons with PG remains elusive. Cross-reactivity between placental tissue and skin has been proposed to play a role. PG has a strong association with HLA-DR3 (61–80 %) and HLA-DR4 (52 %), or both (43–50 %), and virtually all patients with a history of PG have demonstrable anti-HLA antibodies. The placenta is known to be the main source of disparate (paternal) antibodies and can thus present an immunologic target during gestation. …