Publish date: Posted on
Last updated: October 21, 2023
Keywords #
gestational pemphigoid
pemphigoid gestationis
herpes gestationis
bullous dermatosis of pregnancy
Diagnosis #
Pemphigoid gestationis (PG), also known as gestational pemphigoid, is a rare pregnancy-associated blistering disorder that is characterized by pruritic papules, plaques, and bullae which typically presents in late pregnancy.[1] The lesions classically begin in the umbilical and periumbilical region, spread to the abdomen and extremities, and spare the face and mucosa. The pathogenesis of the disease is multifactorial and influenced by hormonal, immunologic, and genetic factors leading to development of the herpes gestationis factor (HGF) antibody, which is a complement-fixing autoantibody to the bullous pemphigoid antigen 2 (BP180). PG also has a strong association with the human leukocyte antigens, HLA-DR3 and -DR4 subtypes.[1,2]
Key Concepts #
- PG is an autoimmune blistering disease associated with pregnancy.
- PG is associated with pregnancy complications, including preterm labor, low birth weight, and neonatal PG.
- Patients must be educated that there is a high rate of recurrence in subsequent pregnancies.
- Many patients have flairs even after delivery.
- First-line treatment is topical corticosteroids, but many patients will require systemic steroids.
Epidemiology #
The incidence of PG is thought to be approximately 1 in 500,000 pregnancies.[1,3] PG has been associated with other autoimmune diseases, including Graves’ disease and Celiac disease.[4,5]
Clinical Features #
- Patients typically present with formation of intensely pruritic, urticarial papules and plaques, and tense vesicles and bullae on the abdomen and upper and lower extremities.[3]
- Periumbilical involvement is classic for PG.
- The disease may have a relapsing and remitting course due to hormonal fluctuations during pregnancy and in the post-partum period.
- Subsequent pregnancies are associated with earlier onset and more severe course, and some patients may note an association with their menstrual periods.[1]
- Maternal complications of PG are rare; however, there is an increased risk of preterm delivery and low birth weight. There is also an increased risk of neonatal PG due to maternal transfer of autoantibodies.[1,3]
Differential Diagnoses #
- Polymorphic eruption of pregnancy (PEP)
- Contact dermatitis
- Bullous pemphigoid
- Erythema multiforme
- Dermatitis herpetiformis
PEP typically starts in the striae whereas PG tends to involve/start in the periumbilical region.
Diagnostic Workup #
The diagnosis of PG is made clinically along with a lesional biopsy for histopathologic examination and a perilesional biopsy for direct immunofluorescence (DIF), as well as serological testing for both indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA). DIF will display linear C3 deposition at the basement membrane zone. Indirect immunofluorescence testing will reveal positive testing for HG factor.
Clinically, umbilical and periumbilical involvement can help differentiate PG from other dermatosis of pregnancy.[1]
Treatment #
Pemphigoid gestationis will resolve on its own even without treatment; however, treatment may improve symptoms, hasten resolution, and reduce complications.[1] Mild disease may be managed with high-potency topical corticosteroids. Widespread disease and cases unresponsive to topical steroids typically require systemic corticosteroids for adequate control. Most patients see improvement in symptoms within days of starting systemic steroids. Patients with disease refectory to systemic steroids may be tried on other immunosuppressive agents.[3]
Slide Viewer #
https://utahderm.med.utah.edu/image-viewer/References #
- Engineer L, Bhol K, Ahmed AR. Pemphigoid gestationis: A review. Am J Obstet Gynecol. 2000. doi:10.1067/mob.2000.105430
- Shornick JK, Jenkins RE, Briggs DC, et al. Anti-HLA antibodies in pemphigoid gestationis (herpes gestationis). Br J Dermatol. 1993. doi:10.1111/j.1365-2133.1993.tb11843.x
- Lipozenčić J, Ljubojevic S, Bukvić-Mokos Z. Pemphigoid gestationis. Clin Dermatol. 2012;30(1):51-55. doi:https://doi.org/10.1016/j.clindermatol.2011.03.009
- Shornick JK, Black MM. Secondary autoimmune diseases in herpes gestationis (pemphigoid gestationis). J Am Acad Dermatol. 1992. doi:10.1016/0190-9622(92)70081-P
- Saffari H, Zone JJ, Allen M, Leiferman KM. A subset of patients with pemphigoid (herpes) gestationis has serological evidence of celiac disease. Int J Dermatol. 2018;57(5):534-540. doi:10.1111/ijd.13925
