Gestational pemphigoid

Orphanet J Rare Dis. 2014 Sep 2:9:136. doi: 10.1186/s13023-014-0136-2.

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.

Publication types

  • Review

MeSH terms

  • Autoantigens / blood
  • Biopsy
  • Collagen Type XVII
  • Diagnosis, Differential
  • Female
  • Fluorescent Antibody Technique, Direct
  • Humans
  • Infant, Newborn
  • Non-Fibrillar Collagens / blood
  • Pemphigoid, Bullous / complications
  • Pemphigoid, Bullous / diagnosis*
  • Pregnancy
  • Pregnancy Complications / diagnosis*
  • Pregnancy Complications / pathology
  • Pregnancy Outcome
  • Prognosis

Substances

  • Autoantigens
  • Non-Fibrillar Collagens