Pemphigoid gestationis

Pemphigoid gestationis is a severe bullous eruption that occurs in 1 in 60,000 pregnancies.

It is unusual for a primigravid women to be affected. In affected women the skin lesions become progressively more severe during successive pregnancies.

• self-limited autoimmune bullous disorder that presents mainly in late pregnancy or the immediate postpartum period but can appear in any of the three trimesters
• beyond pregnancy it can also very rarely occur in association with trophoblastic tumours (choriocarcinoma, hydatidiform mole)
• also an increased risk to develop other autoimmune diseases, in particular Grave's disease
• pemphigoid gestationis tends to recur in subsequent pregnancies, with usually earlier onset and increasing severity. Only very rarely (5%) a pregnancy may be passed over

Pemphigoid gestationis presents with intense pruritus that occasionally may precede the manifestation of skin lesions

• initially, erythematous urticarial papules and plaques typically develop on the abdomen
  • in this so-called pre-bullous stage, differentiation between pemphigoid gestationis and polymorphic eruption of pregnancy is almost impossible, both clinically and histopathologically
• occurs during the second and third trimesters of pregnancy
  • initial sites include the periumbilical area, around the mouth, palms and soles
  • later lesions may become widespread
  • after about 4 weeks bullous lesions appear bullous lesions resolve about 4 weeks post-partum

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Risk for foetus;

• increase in prematurity and small-for-date babies
• neonatal pemphigus is a rare complication of pregnancy in pemphigus patients

Treatment

• seek expert advice
• depends on the stage and severity of the disease and aims to control pruritus and to prevent blister formation
  • in cases of mild pre-blistering pemphigoid, topical corticosteroids with or without oral antihistamines may be sufficient
  • all other cases require systemic corticosteroids
  • severe cases may benefit from immunopheresis
  • after delivery, if necessary, other immunosuppressive treatment may be used

Reference:

1. Himeles JR, Pomeranz MK. Recognizing, Diagnosing, and Managing Pregnancy Dermatoses. Obstet Gynecol. 2022 Oct 01;140(4):679-695.