Gestational Pemphigoid-From Molecular Mechanisms to Clinical Outcomes: A Case Report and Review of Literature.

Abstract

Gestational pemphigoid is a rare, autoimmune, subepidermal bullous disease with an incidence of 1 in 50,000 pregnancies, displaying itself through pruritic erythema and urticarial papules and plaques that evolve into tense bullae. Histopathological findings consist of subepidermal vesicles with perivascular eosinophils and lymphocytes, and direct immunofluorescence reveals C3 complement and, more rarely, IgG in a linear band along the basement membrane. The course is usually self-limiting within 6 months after delivery but, later, can be triggered by subsequent pregnancies, menstruation, or treatment with oral contraceptives. The newborn can be affected due to the transplacental passage of the maternal immunoglobulins, but, usually, less than 10% of newborns will develop lesions similar to pemphigoid gestationis. The diagnosis and management pose a difficult challenge and should be guided by the severity of the disease. We, therefore, provide a short literature review and discussion plus a case from our clinic, with a typical presentation but a delayed diagnosis and an undulating evolution, with severe manifestations and particularly difficult management due to unexpected complications.