[Pemphigoid gestationis: a review]

Abstract

Background: Pemphigoid gestationis (PG) is a rare auto-immune bullous disease occurring in one pregnant woman over 20,000 to 50,000.

Objective: Review of literature about physiopathology, immunological diagnosis and treatment of PG.

Methods: Research on Medline and Embase database without any time limit until April 2010. Because of the lack of randomized therapeutic trials in PG, retrospective series and case reports have been analyzed.

Results: PG is due to auto-antibodies directed against BP180 secondary to a mother-fetus immunological tolerance breaking. Blister formation results from a complex mechanism involving TH2 lymphocytes, cytokines and polymorphonuclear cells. Clinically, the disease is characterized by a pruritic, more or less extensive erythemato-papulous eruption. The presence of vesicles and/or blisters is inconstant but evocative. Recovery occurs generally in a few weeks after delivery but relapses are frequent in subsequent pregnancies. Combined oral contraception may also trigger flares of the disease. Diagnosis is confirmed by direct immunofluorescence showing linear C3±IgG deposits in the dermal-epidermal junction. ELISA BP180-NC16A method is very sensitive to detect circulating auto-antibodies. Fetal prognosis is good, but early onset in 1(st) or 2(nd) trimester and blister formation are risk factors for prematurity and low birth weight. Rarely the newborn may be affected by very transitory blisters. Efficiency of very potent topical corticosteroids has been showed in several studies, and they may be used as first-intent treatment in moderate forms of PG. Systemic corticosteroids are indicated in extensive forms. In very exceptional chronic or relapsing PG, immunosuppressant agents may be necessary.

Conclusion: In the absence of consensus between moderate and severe forms of the disease, precise modalities of the steroid treatment (topical or systemic, duration and rhythm of decrease) are not well defined.