Herpes Gestationis:
A Case Presentation

Robert A. Norman, D.O., MPH, FAAIM and Dyan J. Harvey-Dent, D.O.

Case Presentation

A 32-year-old female at the 20th week gestation (gravida 4, para 4) presented to the office complaining of an intensely pruritic rash that began two weeks ago on her abdomen and had progressed to her breasts and arms, sparing the face, palms and feet. Generalized small vesicles, tense bullae and secondary lesions of excoriation were noted. The patient denied any fever, chills, nausea or vomiting. Patient had never developed this type of rash with her previous pregnancies. The patient’s past medical history was unremarkable. Previous surgical history was only significant for a cesarean section. Current medications included cephalexin and hydroxyzine.

An assessment of Herpes Gestationis was established. To confirm this diagnosis, three punch biopsies of normal and involved skin were done and sent for histologic and immunopathologic exams. The patient was treated with prednisone 20mg/day, topical corticosteroids and diphenhydramine. The patient returned to the office for a one week follow-up with much improved signs and symptoms. The biopsy report confirmed the diagnosis of Herpes Gestationis. The prednisone was then tapered and discontinued.

Discussion

Herpes Gestationis (pemphigoid gestationis) is a rare (1 in 50,000 pregnancies) autoimmune antibody-mediated disease that occurs either during pregnancy or the postpartum period.^2,3 The name of the disease is misleading because Herpes Gestationis has no association with the herpes virus infection.

The onset of disease is usually during the second or third trimester (average 21 weeks gestation). The rash initially appears as edematous, erythematous, annular or polycyclic plaques, appearing in crops with tense vesicles and bullae on the abdomen and extremities, and coalesce rapidly to also involve the back and chest. Usually the face, oral mucosa, palms and soles are spared. Pruritis is intense. Duration of the lesions is variable. Seventy-five percent of patients will have a flare at delivery, but typically spontaneous resolution occurs within three months postpartum.^2,4

Herpes Gestationis may occur for the first time during any pregnancy, but once it has occurred, it tends to reappear in subsequent pregnancies earlier and more severely. There also may be recurrences with the use of oral contraceptives or with menses leading to a protracted course; “conversion to Bullous Pemphigoid.”^3,4 Herpes Gestationis may also occur in association with hydatidiform mole and choriocarcinoma.^1,3,4

The etiology of Herpes Gestationis remains uncertain. There is evidence that supports Herpes Gestationis as an autoimmune process. There is a genetic predisposition with 90% of patients expressing class II antigens [alleles HLA-DR3 (61-80%), HLA-DR4 (52%) or both (43-50%)] and most carry a class III antigen (C4 null allele).4 Herpes Gestationis appears to be mediated by an Ig-G1 subclass and the antigenic target is a 180-kd hemidesmosomal glycoprotein which is the bullous pemphigoid antigen (BPAg2).^2,4 African American women rarely manifest Herpes Gestationis.⁴ This is theorized to be secondary to the low incidence of HLA-DR4 in African Americans.⁴ There is also an increased risk of developing Graves Disease in patients with a history of Herpes Gestationis.¹ There is no other maternal health risks in Herpes Gestationis.

Herpes Gestationis has been associated with prematurity and small-for-gestational-age neonates, but without any increased fetal morbidity and mortality.³ The newborn fetus will have cutaneous involvement approximately 10% of the time, most likely secondary to passive transfer of Herpes Gestationis antibody.^2,3,4 The cutaneous eruption is self-limited and resolves spontaneously within days to weeks.

Upon histopathologic exam of the bullous lesions, you see subepidermal edema and inflammatory dermal infiltrate with eosinophils and spongiosis.⁴ The characteristic direct immunofluorescence feature is a linear bandlike deposit of C3 along the basement membrane zone with concurrent IgG deposition.^2,4

Oral corticosteroids with starting dosages of 20-40mg/day are usually required for control.^1,2,3,4 The dosage is then gradually tapered. The use of topical corticosteroids are helpful for mild cases. Pyridoxine has been reported to have helped. Azathioprine has also been used for disease that is steroid-dependent or steroid-resistant.¹ Case studies have indicated some benefit from tetracyclines in postpartum Herpes Gestationis, but their effectiveness requires further investigation.⁴

 

© 2005 American Association of Physician Specialists, Inc.

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