Pemphigoid Gestationis Clinical Presentation

Updated: Jun 02, 2017
  • Author: Victor A Teran; Chief Editor: William D James, MD  more...
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Presentation

History

Pemphigoid gestationis typically manifests during late pregnancy, with an abrupt onset of extremely pruritic urticarial papules and blisters on the abdomen and trunk. Unrelenting pruritus often interferes with daily activities.

Lesions may appear any time during pregnancy, but they most commonly develop during the second and third trimesters.

Symptoms may abate at the end of pregnancy; however, dramatic flares can occur at or immediately after delivery. Pemphigoid gestationis usually resolves spontaneously within weeks to months after delivery and possibly quicker with breastfeeding. The persistence of disease activity for years postpartum has been reported.

Pemphigoid gestationis may recur with the resumption of menses, use of oral contraception, and subsequent pregnancies. The 1999 cohort study by Jenkins et al [8] showed no association between change in partner and development of pemphigoid gestationis in subsequent pregnancies.

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Physical Examination

The initial clinical manifestations are erythematous urticarial patches and plaques, which are typically periumbilical; this is distinct from the presentation of pruritic urticarial papules and plaques of pregnancy, another dermatosis of pregnancy that spares the umbilicus. [9] These lesions progress to tense vesicles and blisters. However, some patients may present with urticarial plaques and may never develop blisters (see the images below). These hivelike plaques differ from true urticaria because of their relatively fixed nature. The rash spreads peripherally, often sparing the face, palms, and soles. Mucosal lesions occur in less than 20% of cases. Patients may have secondary infections at blister sites.

Tense bullae are present on the arms of this other Tense bullae are present on the arms of this otherwise healthy 32-year-old primigravida woman.
Urticarial or hivelike plaques, as seen on the pos Urticarial or hivelike plaques, as seen on the posterolateral neck of this woman in her third trimester, can be observed in patients with pemphigoid gestationis.
A close-up view of a blister reveals the tense pri A close-up view of a blister reveals the tense primary lesion filled with clear fluid.
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Causes

Pemphigoid gestationis is a pregnancy-associated autoimmune disease. The autoantibodies are deposited in the skin and detectable in the circulation, and they are predominantly specific for the hemidesmosomal protein BPAG2.

Circulating antibodies and T cells are directed against an immunodominant epitope. This epitope, located in the extracellular region of BPAG2 near the membrane, is called the MCW-1 domain. This region of BPAG2 is also an immunodominant epitope in a closely related autoimmune blistering disease, bullous pemphigoid.

The trigger for autoantibody production is still poorly understood. As described in Pathophysiology, autoantibodies to amniotic basement membrane (paternal major histocompatibility class II antigens) may cross-react with BPAG2 antigen in the skin, leading to the immune response.

Pemphigoid gestationis has also been described to occur in association with trophoblastic tumors, such as hydatiform mole or choriocarcinoma.

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Complications

Women with pemphigoid gestationis have increased incidences of premature delivery and SGA neonates. An increased lifetime risk of other autoimmune conditions, such as Graves disease, is documented.

Infants born to affected women rarely have transient blistering disease. These infants are at risk for infection, thermoregulatory difficulties, and fluid and electrolyte abnormalities.

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