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Review
. 2014 Sep 2:9:136.
doi: 10.1186/s13023-014-0136-2.

Gestational pemphigoid

Affiliations
Review

Gestational pemphigoid

Laura Huilaja et al. Orphanet J Rare Dis. .

Abstract

Gestational pemphigoid (pemphigoid gestationis, PG) is a rare autoimmune skin disorder occurring characteristically during pregnancy. Autoantibodies against placental BP180 (also known as BPAG2 or collagen XVII) cause damage to the skin basement membrane, resulting in severe itching and blistering rash over the body and the extremities. The diagnosis of PG is confirmed by immunofluorescence analysis of a skin biopsy, while serum levels of pemphigoid antigen BP180 antibody can be used to assess disease activity. PG with mild symptoms can be treated with topical corticosteroids, while oral corticosteroids are the mainstay in treatment of severe PG. PG usually flares up at the time of delivery, and resolves spontaneously shortly after. However, relapses in subsequent pregnancies are common. As PG has been linked to the risk of prematurity and fetal growth restriction, prenatal monitoring jointly by a dermatologist and an obstetrician is recommended. Mothers should also be informed of the potential risk of re-activation of the disease in subsequent pregnancies and during hormonal contraception.

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Figures

Figure 1
Figure 1
Skin findings of gestational pemphigoid (PG). Urticarial papules and plaques usually appearing first on abdominal area (A). Minor umbilical lesions of PG (B). Vesicles (C) and bullae (D) following urticarial plaques. PG lesions on extremities (E-G).
Figure 2
Figure 2
Linear complement 3 (C3) (arrow) fluorescence in immunofluorescence analysis of perilesional skin biopsy is diagnostic for gestational pemphigoid. Primary magnification 200 × .

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