Pemphigoid gestationis.

Pemphigoid gestationis is a rare autoimmune skin disorder emerging exclusively during pregnancy. Topical and oral glucocorticoids as well as oral antihistamines are the standard medications administered during pregnancy, aiming to relieve pruritus and to suppress extensive blister formation. Obstetricians should be able to recognize and treat this rare clinical condition accordingly.

Question

What is this condition and how it should be treated?

Answer

A 27‐year‐old patient at 34‐week gestation (para 1, gravida 1) presented at the Emergency Room complaining of blisters and urticarial lesions. Clinical examination and subsequent immunofluorescent skin biopsy revealed pemphigoid gestationis (PG). The patient was treated with systemic corticosteroids and local application of mometasone furoate. Cesarean section was performed at 39th gestational week, with delivery of a live fetus weighing 3240 g. Treatment was maintained until 5 weeks after delivery. PG is a rare (1:50,000) pruritic autoimmune skin disorder emerging exclusively during pregnancy. Patients usually present during the second and third trimester. PG tends to recur in subsequent pregnancies with earlier onset and a more severe course. Lesions start around the umbilicus (Fig. 1) and then become widespread (Fig. 2). Autoantibodies form against BP180 (also known as BPAG2 or collagen XVII), resulting in the destruction of the skin membrane. Potent topical glucocorticoids, oral glucocorticoids, and oral antihistamines are the standard medications that are used to treat PG during pregnancy. However, plasmapheresis and immunophoresis have also been used during pregnancy. In the postpartum period, topical and oral glucocorticoids continue to be the treatments of choice. However, other treatments that have been tried in isolated cases include tetracyclines with or without nicotinamide, cyclophosphamide, plasmapheresis, intravenous immunoglobulin, rituximab, and goserelin 1.

Figure 1

Blisters and urticarial lesions around the umbilicus.

Figure 2

Blisters and urticarial lesions located in the abdomen.

Authorship

IKP and ST: involved in treatment of the patient in the Emergency Room. IKP, CGL, AB, PK, KN, and EP: involved in treatment of the patient in the Hospital. NV: prepared figures. IKP, PK, and NT: edited and revised the manuscript. PK, NT, and DL: approved final version of the manuscript.

Conflict of Interest

None declared.