Two Cases of Herpes Gestationis

Abdominal rash in a primigravida. A 24-year-old woman's first pregnancy was uneventful until the sixth month when mild malaise and a highly pruritic abdominal rash occurred (A). A biopsy and direct immunofluorescence confirmed the suspected diagnosis of herpes gestationis.

Herpes gestationis (or pemphigoid gestationis) is an autoimmune, bullous disorder of pregnancy that can also occur in patients with hydatidiform mole and choriocarcinoma. The skin protein targeted in herpes gestationis is among those involved in bullous pemphigoid. Most affected patients are white, which is probably related to the disease's association with certain HLA antigen haplotypes.¹

The onset of herpes gestationis is usually in the second trimester, but the disorder may not occur until the early postpartum period. Cutaneous erythema, edema, and pruritus are characteristic; a prodrome of flu-like symptoms has been reported.² The plaques can occur anywhere on the body but have a predilection for the abdomen, especially the area of the umbilicus. Mucous membranes are rarely involved. Papules, vesicles, and bullae begin to appear, often in a polycyclic pattern, within a few days. The presence of vesicles and bullae helps differentiate herpes gestationis from other disorders that may occur in pregnancy, such as erythema multiforme, drug eruptions, or pruritic and urticarial papules and plaques of pregnancy. A leukocytosis may be present. A biopsy, as well as direct immunofluorescence, can help confirm the diagnosis.

Herpes gestationis tends to wax and wane, and outbreaks may occur for months after delivery. Often a major flare erupts at the time of delivery, even if the disease has been under control. Postpregnancy episodes may be related to menses or to the initiation of oral contraceptives. Outbreaks in subsequent pregnancies are common and are often more severe and of earlier onset.

The relationship between herpes gestationis and stillbirth is being investigated³; the disease has been associated with the birth of premature and small-for- gestational-age infants.⁴ The neonate with cutaneous involvement typically has mild, transient disease that clears without treatment; this spontaneous resolution may be attributed to clearance of the maternal HG factor, an IgG antibody. If necessary, topical corticosteroids and antihistamines can be given. No long-term sequelae have been reported in the children of women with herpes gestationis.⁵

Oral antihistamines and high-potency topical corticosteroids were prescribed for this patient, whose relatively mild disease occurred intermittently throughout the remainder of her pregnancy. A flare at childbirth gradually faded over several months; the newborn was not affected.

Hivelike lesions in a teenager. During the 38th week of her first pregnancy, a 16-year-old patient with no history of systemic illness was affected with pruritic, hivelike, edematous bullous lesions on her abdomen and legs (B). At 40 weeks' gestation, a healthy child was delivered; the neonate was free of lesions and continued to develop normally.

Herpes gestationis was diagnosed; this dermatosis is not associated with any herpes viruses. The disorder appears to be induced by an IgG antibody (the HG factor) specific for the epidermal basement membrane zone.

Herpes gestationis can be confused with erythema multiforme. The latter condition typically features target lesions of the extremities, whereas herpes gestationis lesions are commonly centered in the umbilical area where the skin itches and burns intensely. Histologic studies and immunofluorescence help confirm the diagnosis. In herpes gestationis, the subepidermal blister has eosinophils within the blister fluid and an edematous upper dermis with a mixed infiltrate of lymphocytes, macrophages, and eosinophils. Spongiosis and necrotic keratinocytes also may be seen. In nearly all cases, immunofluorescence demonstrates complement deposition along the basement membrane zone, which is not seen in erythema multiforme. These findings also help rule out urticaria, bullous pemphigoid, and pruritic urticarial papules and plaques of pregnancy.

This patient's lesions became larger and more symptomatic after her pregnancy. Two weeks postpartum, she presented with multiple, 1- to 15-cm, erythematous, urticarial plaques localized to the periumbilical abdomen and lower trunk. These lesions appeared more urticarial; the older and larger lesions had vesiculation and firm bullae. Some showed central clearing with a thin, adherent scale. Smaller plaques and bullae were present on the upper and lower extremities. Prednisone, 40 mg/d, was given until the lesions resolved 4 weeks after delivery. (The suture at the biopsy site is visible next to the umbilicus.)

(First case and photograph courtesy of Neil F. Gibbs, MD. Second case and photograph courtesy of Scott Ross, MD.)

References:

REFERENCES:

1.

Shornick JK, Meek TJ, Nesbitt LT Jr, Gilliam JN. Herpes gestationis inblacks. Arch Dermatol. 1984;120:511-513.

2.

Winton GB, Lewis CW. Dermatoses in pregnancy. J Am Acad Dermatol.1982;6:977-998.

3.

Kolodny RC. Herpes gestationis. A new assessment of incidence, diagnosis,and fetal prognosis. Am J Obstet Gynecol. 1969;104:39-45.

4.

Holmes RC, Black MM. The fetal prognosis in pemphigoid gestationis(herpes gestationis). Br J Dermatol. 1984;110:67-72.

5.

Shornick JK. Herpes gestationis. J Am Acad Dermatol. 1987;17:539-556.