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.
Highlights
Gestational pemphigoid is a rare autoimmune skin disorder that occurs during pregnancy
If Pemphigoid gestationis (PG) is suspected, measurement of serum Bullous pemphigoid antigen 180 (BP180) antibody level is recommended, as it correlates with the degree of disease severity and facilitates assessment of treatment response [33,34]
Previous studies have demonstrated that in the treatment of bullous pemphigoid (BP) the use of oral prednisolone is associated with more frequent severe adverse events and increased mortality compared to topical corticosteroids [1,30,42]
Summary
Gestational pemphigoid (pemphigoid gestationis, PG) is a rare autoimmune skin disorder that occurs during pregnancy. Previous studies have demonstrated that in the treatment of BP the use of oral prednisolone is associated with more frequent severe adverse events and increased mortality compared to topical corticosteroids [1,30,42]. Fetus and the newborn The risk of preterm birth and fetal growth restriction is greater in PG pregnancies compared to normal population [57,58,59,60]. Among 12 Finnish PG patients increased umbilical artery pulsatility was detected only in one pregnancy with pre-eclampsia and fetal growth restriction; all other PG pregnancies showed normal umbilical artery blood flow findings and biophysical scores [13], suggesting that clinically significant placental failure is rare in PG. Proneness to Hashimoto’s thyroiditis, autoimmune thrombocytopenia and pernicious anemia has been reported to be increased [16,67]
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