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Pemphigus Vulgaris in Pregnancy

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We report the case of a 34-year-old woman who was diagnosed with pemphigus vulgaris (PV) during pregnancy. The patient presented with widespread blistering dermatitis and associated burning and pruritus. At 6 weeks’ gestation the patient was admitted to the hospital to expedite her diagnosis and initiate treatment. A skin biopsy revealed suprabasal acantholysis, and direct immunofluorescence demonstrated diffuse intercellular IgG in the epidermis and basal intercellular C3, which confirmed the diagnosis of PV. Treatment with corticosteroids was instituted after discussions with the patient about possible adverse effects to the fetus. Pemphigus vulgaris is rare in pregnancy and active PV presents potential threats of fetal spread and transient lesion production, which is associated with increased mortality and morbidity in the fetus. Our patient had active PV and required treatment throughout her pregnancy. The pregnancy progressed to premature delivery of the neonate without skin lesions or apparent complications.

Practice Points

  • ­Early diagnosis and appropriate treatment of pemphigus vulgaris in pregnancy is paramount in protecting the health of the mother and fetus.
  • Management of autoimmune diseases during pregnancy continues to present numerous challenges for physicians due to the pathology of the diseases as well as the sensitive nature of pregnancy and lack of robust data in this patient population.


 

References

Pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis that has not shown a predilection toward a particular race or sex.1 Autoantibodies for desmoglein 1 and desmoglein 3, members of the cadherin family that are involved in cellular adhesion, have been linked to the pathogenesis of PV.2 These autoantibodies play a role in the loss of cell-to-cell adhesion in the basal and suprabasal layers of the deep epidermis while cellular adhesion in the superficial epidermis remains intact, leading to the clinical presentation of epidermal blistering and ulcerations most commonly found on the scalp, face, groin, and axillae. Diagnosis typically is made based on skin biopsy and confirmed by direct immunofluorescence. Histologically, PV displays acantholysis and suprabasal cleft formation. Immunofluorescence may show IgG antibodies against the PV antigen in the epidermis.3 Once a diagnosis has been made, treatment typically consists of systemic steroids, as the use of steroids has had great effect in preventing infections, sepsis, and fatality that were once associated with PV.4 Mortality rates associated with PV have decreased to 10% to 15% with systemic steroids from a mortality rate as high as 70% in the presteroid era.1,5 Treatment of PV during pregnancy, as in our patient, requires obstetric and pediatric consultations before therapy is initiated. Use of corticosteroids during pregnancy can be potentially dangerous to the fetus, particularly if high doses are necessary to control maternal disease.6,7

Case Report

A 34-year-old pregnant woman at 6 weeks’ gestation presented with widespread blistering dermatitis and associated burning and pruritus. Her obstetrical history was gravida 3, para 2. The patient reported a “rash” on the scalp that had developed 9 months prior. She had been treated as an outpatient at an outside institution with topical antibiotics and antifungal medications, yet the dermatitis progressed. Three weeks prior to hospitalization, the rash was present on the skin and mucosal surfaces, including the groin, chest, face, hard palate, buccal mucosa, lips (Figure 1), and back (Figure 2). Nontender bullae ruptured after 3 days, releasing clear, yellow, serous fluid with associated burning and pruritus. The bullae were hemorrhagic and erythematous at the base.

Figure 1. Facial involvement with bullae, crusted hemorrhagic lesions, and eschar in a 34-year-old pregnant woman.

Figure 2. Involvement of the back with bullae in various stages. Some bullae were intact while others newly erupted.

Figure 3. Superinfected and flaking scalp.

Figure 4. Biopsy revealed suprabasal acantholysis with a tombstone effect of residual basal cells (H&E, original magnification ×200).

At the current presentation, the patient had several excoriated 1- to 2-cm oval denudations; some were crusted with eschar. Nikolsky sign was negative. Multiple confluent bullous lesions had erupted on the entire scalp with a thick, impetiginous, yellow crust. She had a wet, boggy, foul-smelling, superinfected scalp that was mildly tender to touch with flaking tissue debris (Figure 3). A white blood cell count was 13.2×109/L (reference range, 4.5–11.0×109/L) with 5% eosinophils (reference range, 2.7%). The differential diagnosis included bullous impetigo, pemphigoid, Stevens-Johnson syndrome, dermatitis herpetiformis, and pemphigus vulgaris.

Biopsies of the scalp and back were taken and showed suprabasal acantholysis with a tombstone effect of residual basal cells standing up on the basement membrane without the characteristic acantholysis into skin appendages (Figure 4). The acantholytic cells in the bullous chamber did not round up as in Hailey-Hailey disease nor was there the dyskeratosis of Grover disease. Direct immunofluorescence on an elbow punch biopsy found diffuse 1+ intercellular IgG in the epidermis and diffuse 1+ basal intercellular C3, and was negative for IgA, IgM, and C1q, thus confirming a diagnosis of PV.

The patient was started on prednisone 20 mg once daily. An increase to prednisone 60 mg led to initial improvement of symptoms, but there was a relapse after several days, which is typical of PV in pregnancy,7 prompting the dose to be increased to 120 mg. Following alleviation of symptoms, the dose was later tapered back to 60 mg. No lesions were present at discharge or for 2.5 months thereafter, as the prednisone was tapered from 60 to 45 mg daily after discharge.

On follow-up, the patient’s PV was well controlled, but the prednisone dose was back up to 60 mg daily because of 2 new skin lesions that had developed since her last visit 2.5 months prior. Ultrasonography showed no fetal abnormalities as the pregnancy progressed to 28 weeks’ gestation. The patient developed hypertension and went into premature labor due to placenta previa. The neonate showed no skin lesions or anomalies while in the neonatal intensive care unit. The mother’s prednisone dose was tapered from 60 to 20 mg daily while the white blood cell count was 7.1×109/L with 2% eosinophils and a new scalp lesion appeared. Seven months after her initial discharge from the hospital for the dermatologic condition, she was no longer nursing and azathioprine was added to prednisone 60 mg daily.

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