Urticarial plaques with a preference for the abdomen and periumbilical area are typical.

PEMPHIGOID GESTATIONIS

Pemphigoid gestationis (PG), previously known as herpes gestationis, is an autoimmune blistering disorder most commonly associated with pregnancy although it may occur in association with trophoblastic tumors, hydatidiform mole or choriocarcinoma. PG binds the NC16A domain of collagen XVII (BPAG2, BP180).

In a series of 87 patients, the average age was 28 years and the onset ranged from 5 weeks gestation to 35 days postpartum. 18% presented in the first trimester, 34% in the second, 34% in the third and 14% presented postpartum. In that same review, there was no association with change in male partner as previously suggested.
Most patients flare with delivery.
PG is associated with adverse pregnancy outcome (e.g. miscarriage, preterm delivery, fetal growth restriction, anhydramnios and blisters in neonate). Of note, collagen XVII is predominantly expressed in the skin, but may also be present in the amniotic epithelia, placenta and amniotic fluid.
One woman had two episodes triggered by spontaneous abortion (presented with urticarial rash and heavy vaginal bleeding) but had 3 normal pregnancies in between.

Itching is often the first symptom. The skin lesions of PG often begin on the abdomen and include urticarial plaques, vesicles and frank bulla. It may then spread to the rest of the trunk and extremities, but is rare on the face or oral mucosa. Flares have been noted after delivery, with the resumption of menses, ovulation or beginning oral contraceptives. Approximately 20% of patients will experience premature delivery. Up to 10% of the babies will develop skin lesions due to passive transfer of antibodies. Approximately 10% of patients will develop Graves disease.

Pemphigoid Gestationis vs PUPPP

Characteristic	Pemphigoid Gestationis	PUPPP
Onset	1 trimester thru postpartum	3rd trimester
Gravida	Multigravida	Primigravida
Location preference	Periumbilical	Stria
Morphology	Urticarial plaque → Vesiculobullous; targetoid	Papulourticarial
Pathogenesis	Autoimmunity	Stretching eliciting immune response
Recurrence	Common	Rare
Baby	SGA, prematurity; fatality reported	Rare problems

Immunoflourescence

DIF of perilesional skin shows linear deposition of C3 along the basement membrane zone. Alternatively CD4 immunohistochemistry of Formalin fixed tissue is positive in PG and negative in PUPPP. Enzyme-linked immunosorbent assay (ELISA) detects the specific antibodies (usually IgG4) against collagen XVII.

BP180 ELISA is a valuable test to differentiate PG from PUPPP as it is highly sensitive for PG, negative in PUPPP and is unaffected by the use of topical steroids which can cause a skin biopsy DIF to give a false negative result [JAAD 2017;76;560].

Neonatal Pemphigoid

Maternal antibodies may be transferred to the fetus resulting in vesicles and bulla in the newborn.

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