M. Daboubi, T. Maaita, M. Al-Ruhaibeh
M. Daboubi, T. Maaita, M. Al-Ruhaibeh
M. Daboubi, T. Maaita, M. Al-Ruhaibeh
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Herpes Gestationis: A Case Report<br />
Moh’d K. <strong>Daboubi</strong> MD*, Tagreed J. <strong>Maaita</strong> MD**, Maysoon K. <strong>Al</strong>-<strong>Ruhaibeh</strong> MD^<br />
ABSTRACT<br />
We report a case of a 34 year old pregnant female patient who presented with herpes gestationis in her second<br />
trimester. She was gravida 5, para 4 with skipped or uninvolved pregnancies and was managed at Queen <strong>Al</strong>ia<br />
Military Hospital. This autoimmune bullous dermatosis is very rare and usually persists during the postpartum<br />
period.<br />
Key words: Herpes Gestations, Pregnancy, Histopathology<br />
JRMS July 2010; 17(Supp 2): 94-97<br />
Introduction<br />
Herpes gestationis, also known as Pemphigoid<br />
gestations, is a rare recurrent vesiculobullous<br />
autoimmune disease of pregnancy and the<br />
postpartum period. Most cases resolve within few<br />
months of delivery. The disease is characterized by<br />
intense pruritic skin eruptions and may result in<br />
increased fetal morbidity. Its incidence ranges from<br />
1:50,000 to 1:500,000 pregnancies. (1) The<br />
interaction of this rare pathology with pregnancy is<br />
underestimated by obstetricians. (2)<br />
Case Report<br />
A 34-year-old Jordanian lady gravida 5, para 4, all<br />
her previous pregnancies ended in normal vaginal<br />
deliveries. The patient attended King Hussein<br />
Medical Centre at nine weeks gestation and had<br />
regular follow up for a singleton pregnancy. At 26<br />
weeks gestational age she suddenly developed<br />
severe intensely itchy urticarial papules and plaques<br />
which later became vesiculobullous lesions, starting<br />
mainly around the umbilicus (Fig. 1) and then<br />
spreading to involve the whole body (Fig. 2) sparing<br />
the face, mucous membranes, palms and soles<br />
When the patient’s history was reviewed it was<br />
noted that she developed the same lesions in her<br />
second and fourth pregnancies, both started in the<br />
second trimester around the 26 th week of gestation<br />
and developed when she took oral contraceptive<br />
pills after her third pregnancy. During each attack<br />
she was admitted and a skin biopsy was taken and<br />
herpes gestationis was confirmed. She was treated<br />
with prednisolone tablets with a good response in<br />
few days.<br />
On this admission two-skin biopsies from her left<br />
arm were taken, one from lesional skin for<br />
histopathology examination and the other one from<br />
peri-lesional skin for direct immunoflourescence.<br />
The first one demonstrated sub epidermal vesicles<br />
with focal spongiosis, a heavy perivascular<br />
lymphohistiocytic infiltrate and esinophils in the<br />
dermis and in the bullae (Fig. 3 & 4). Direct<br />
immunoflourescence demonstrated heavy linear C3<br />
deposits while IgG, IgM, IgA and fibrinogen were<br />
negative.<br />
She was started orally on 40mg prednisolone<br />
tablets per day, in addition to topical steroid<br />
ointment and systemic antihistamine. She responded<br />
well to this treatment in few days, and was<br />
discharged home after eight days on 40mg<br />
prednisolone daily; two weeks later the dose was<br />
gradually decreased to 30mg per day. She attended<br />
From the Departments of:<br />
*Gynecology and Obstetrics, IVF Unit, King Hussein Medical Centre (KHMC), Amman-Jordan.<br />
**Dermatology, Queen <strong>Al</strong>ia Military Hospital (QAMH), Amman-Jordan.<br />
^ Histopathology<br />
Correspondence should be to Dr. M. <strong>Daboubi</strong>, P. O. Box 620812 Amman 11162 Jordan, E-mail: mdaboobi@hotmail.com<br />
Manuscript received April 3, 2006. Accepted August 31, 2006<br />
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Vol. 17 Supp No. 2 July 2010
Fig. 1. Pruritic urticarial lesions developing a periumbilical<br />
pattern on the abdomen<br />
Fig. 2. Annular erythema on the fore arm<br />
Fig. 3. Dense peri-vascular dermal lymphohistiocytic<br />
infiltrate and esinophils<br />
antenatal clinic and the dermatology clinic as well<br />
regularly every two weeks.<br />
She delivered vaginally at 39 weeks a baby boy<br />
weighing 3.150 kilograms who was healthy without<br />
abnormalities or skin lesions. She had two very<br />
small skin lesions at time of delivery.<br />
The patient experienced a postpartum flare-up on<br />
the third day while she was still in the hospital;<br />
prednisolone dose was increased to 50mg per day<br />
and she was discharged five days later on 40mg per<br />
day. Later the dose was decreased gradually till it<br />
was stopped at the 14 th week post partum.<br />
It is important to notice that the first and the third<br />
pregnancies were free of the disease.<br />
Discussion<br />
Herpes gestationis is one of the specific<br />
dermatoses of pregnancy like Papular dermatitis of<br />
pregnancy. It is characterized by intense pruritic<br />
urticarial papules and plaques with the development<br />
of tense vesicles and bullae, and it tends to recur<br />
with subsequent pregnancies and with the use of the<br />
Fig 4. Sub epidermal vesicle containing lymphohistiocytes<br />
and esinophils<br />
oral contraceptive pill. Herpes gestationis is clearly<br />
hormonally modulated, since the rash flares<br />
premenstrually, (3) however skipped or uninvolved<br />
pregnancies occur in 8% of reported cases. (4,5) The<br />
same occurred with our patient, which is important,<br />
because this is very rare and explanation of why<br />
skipped pregnancies occur remains uncertain. It is<br />
not due to the mother and the fetus being compatible<br />
at the DR locus (as there is an association of Herpes<br />
gestationis with HLA DR3 and DR4 antigens), nor it<br />
is due to a change in partner. (4)<br />
It tends to occur any time from first trimester to<br />
the immediate post partum period but mostly it<br />
begins in the second or third trimester.<br />
Exacerbation at the time of delivery or post partum<br />
period occurs in 75% of the patients and most<br />
patients recover within 14 weeks after delivery. (6)<br />
Persistence up to 28 months post partum has been<br />
frequently reported, (6,7) and exceptionally long<br />
persistence for eight years has been reported in one<br />
case. (6) Herpes gestationis occurs in pregnancy and<br />
in trophoplastic tumors.<br />
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Herpes gestations must be differentiated from<br />
other pruritic skin diseases such as prurigo<br />
gestationis, impetigo herpetiformis and pruritic<br />
urticarial papules and plaques of pregnancy. (8) The<br />
histopathology of Herpes gestationis shows<br />
edematous papillae with sub epidermal vesicles and<br />
dense dermal eosinophilic infiltrate forming<br />
eosinophilic spongiosis. (4)<br />
Direct Immunofluorecence of peri-lesional skin<br />
demonstrated linear deposits of C3 at basement<br />
membrane; IgG deposits may be found in 40-50% of<br />
reported cases. (4,7)<br />
The cause of Herpes gestationis may be related to<br />
abnormal expression of major histocompatibility<br />
complex class П Ag within the placenta that initiate<br />
an allogenic response to the placenta basement<br />
membrane which cross react with skin. (9,10) This<br />
theory is based on an association of Herpes<br />
gestationis with HLA DR3 and DR4 antigens. (1,11,12)<br />
Fabbri et al. suggested that an inflammatory<br />
infiltrate is involved in the production of<br />
Pemphigoid gestationis bullous lesions assuming<br />
that Th2 cells (T helper type 2 cells) might be<br />
implicated in the very early stages of autoimmune<br />
response and may exercise a broad influence in<br />
blister formation in this disease. (13) Jenkins reported<br />
that 13.8% of patients with Herpes gestations had<br />
associated autoimmune disease. (4)<br />
Our patient delivered a full term baby with no<br />
congenital abnormality or cutaneous lesions and<br />
with a normal birth weight. There is no clear<br />
evidence that Herpes gestationis poses significant<br />
risk to either mother or child, (14) in Jenkins study of<br />
278 cases there was 16% spontaneous abortions and<br />
only 2.8 % of infants had evidence of skin lesions. (4)<br />
Cutaneous involvement of the neonate occurs in 2-<br />
10%. (15) In our case no cutaneous involvement<br />
occurred. Neonates should be evaluated for adrenal<br />
insufficiency when affected mother has received<br />
steroids for prolonged periods. (14)<br />
Systemic steroids are the treatment of choice to<br />
relieve pruritus and to suppress the eruption; in<br />
severe reluctant cases intravenous Immunoglobulins<br />
may be needed for few days to initiate remission.<br />
Chlorpheniramine appeared to suppress pruritus,<br />
also topical steroids help. Azathioprine, dapsone,<br />
pyridoxine are used as adjuvant therapy and one<br />
case was helped with goserelin for continuing<br />
disease several years post partum with only initial<br />
success. (16)<br />
Immunoblotting and ELISA are sensitive tools for<br />
the detection of auto antibodies to bullous<br />
pemphigoid antigen (17) 180 KD in patients with<br />
pemphigoid gestations; the ELISA is useful to<br />
monitor auto antibody serum levels.<br />
Conclusion<br />
Herpes gestationis is a pregnancy specific<br />
dermatosis, which usually recurs with each<br />
pregnancy with more severe course and earlier<br />
onset, but disease-free pregnancies may occur.<br />
The disease usually flares up in the early<br />
postpartum period. Early diagnosis and<br />
management may help to prevent maternal and fetal<br />
complications.<br />
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