Pemphigoid Gestationis – Case Report and Review of Literature

Abstract

Pemphigoid gestationis (PG) is a rare autoimmune bullous skin disorder which usually presents with intense pruritus and urticarial lesions that may evolve into vesicles and tense blisters. In majority of patients, it starts in the second or third trimester of pregnancy and resolves spontaneously after delivery. Lesions appear in the periumbilical area in 90% of patients and rapidly spread centrifugally to other parts of the body. The diagnosis needs to be confirmed by direct immunofluorescence test (DIF) with indirect immunofluorescence test (IIF), ELISA and immunoblot techniques playing role in diagnosis and/or monitoring antibodies level. Mild symptoms of PG can be treated with topical therapy only, but in severe course of the disease the treatment may be escalated to oral corticosteroids. We present an unusual case of PG started 2 weeks after delivery with an updated overview on the epidemiology, pathology, clinical picture, treatment, and complications of the disease.

Update on specific dermatoses of pregnancy

“…It is a rare, pruritic autoimmune bullous disease with clinical and pathological features similar to bullous pemphigoid (BP). Its incidence varies from around 1:50,000 to 1:60,000 pregnancies, and it is more common in multiparous women 23 , 24 .…”

“…It usually recurs in future pregnancies, starting earlier and with a more severe presentation 7 , 24 , 25 . Also, it can relapse with menstruation and during the use of oral contraceptives 18 , 23 – 26 . The literature has reported PG associated with trophoblastic tumors such as hydatidiform mole and choriocarcinoma 7 , 24 .…”

“…Clinically, there is an initial phase in which erythematous and urticarial papules and plaques predominate, followed by vesicles and blisters. Itching is intense and can be disabling 23 . In 90% of the cases, the lesions are initially located in the periumbilical region and spread to the abdomen and limbs 20 , 21 , 23 , 24 .…”

“…Itching is intense and can be disabling 23 . In 90% of the cases, the lesions are initially located in the periumbilical region and spread to the abdomen and limbs 20 , 21 , 23 , 24 . The oral mucosa is rarely affected (15–20%) 26 .…”

Pruritus in Pregnancy

“…The natural course of PG includes remissions and exacerbations during pregnancy, with characteristic flares during delivery or immediately postpartum resolving spontaneously within 4 weeks [ 9 , 59 ]. The disease may also flare after abortion.…”