http://dx.doi.org/10.5272/jimab.2014206.

552
JournalofIMAB
ISSN:1312773X
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Journal of IMAB - Annual Proceeding (Scientific Papers) 2014, vol. 20, issue 6

HERPES GESTATIONIS
1

IvelinaYordanova ,ValentinValtchev ,KossaraDrenovska ,DimitarK.

1

Gospodinov ,SnejinaVassileva
1) DepartmentofDermatologyandVenereology,FacultyofMedicine,Medical
University,Pleven,Bulgaria
2) DepartmentofDermatologyandVenereology,FacultyofMedicine,Medical
University,Sofia,Bulgaria.
investigations were within
normal limits. Histological
PGtypicallyaffectspregnant
examination of a biopsy
ABSTRACT
womeninthesecondorthird specimen from a fresh
Herpes gestationis, also known as pemphigoidtrimester but may appear at
vesicular lesion showed
gestationis (PG) is an extremely rare autoimmune bullous anytimeduringpregnancyor
edemainthedermis,mixed
dermatosisofthegestationandpostpartumperiod.Thedis even during the immediate perivascular inflammatory
easewasoriginallynamedherpesgestationisonthebasisof postpartumperiod.
infiltrate with a pre
the morphological herpetiform feature of the blisters. We
dominance of eosinophils
reporta21yearoldwoman,pregnantinthethirdtrimester,
CASEREPORT:
(Fig. 2). Direct
whopresentedwithapruriticbullouscutaneouseruptionof
We present a 21year immunofluorescence (DIF)
twoweeksduration.Thediseasestartedwitharedplaquein old woman pregnant in the on perilesional skin showed
theabdominalareaaccompaniedbymilditching.Soonafter, third trimester. The disease lineardepositionofIgG(++)
blistersappearedandaffectedalmosttheentirebody.Physical started two weeks before her andC3(++)atthecutaneous
examinationrevealedaprimiparouswomaningoodgeneral admission in the Department basement membrane zone
state,pregnantin36weeksofgestation.Theskinchanges of dermatology with red (BMZ) (Fig. 3). Indirect
affected the abdomen, back of the trunk, upper and lowerplaquesontheabdominalskin, immunofluorescence(IIF)on
extremities, hands and feet. They were manifested by a around the umbilicus, human esophagus substrate
polymorphouseruption,consistingoferythematousurticaria accompanied by itching. revealedcirculatingIgGanti
likeplaques,smalltensevesiclesandmultipleexcoriations. Soonafter,blistersappearedon BMZantibodiesatatiterof

(Fig.

4).
Mucous membranes were not affected. Routine laboratory this background and quickly 1:80
ELISABP180NC16A
examinations were within normal limits. Direct spread to affect almost the
(ELISA kit, MBL, Nagoya,
immunofluorescence(DIF)onperilesionalskinshowedlinear entirebody.
Japan)wasstronglypositive
depositionofIgG(++)andC3(++)atthecutaneousbasement
The

physical
88.2 U/ml (cut off <9
membranezone(BMZ).Indirectimmunofluorescence(IIF)onexamination revealed a
negative; 9 positive).
human esophagus substrate revealed circulating IgG anti woman in good general
Based on the data from the
BMZantibodiesatatiterof1:80.ELISABP180NC16Awas condition, afebrile, pregnant
medicalhistory,theclinical
stronglypositive.ThediagnosisofPGwasconfirmedandaatthe36weeksofgestation. featuresandtheresultsfrom

and
treatmentwithsystemicmethylprednisolone60mg/daywas Cardiovascular
the laboratory studies,the
initiated,latergraduallytaperedto20mg/day,togetherwith respiratory systems were diagnosisPGwasconfirmed.

pathological
topical corticosteroids. As a result on the 10th day of the without
Treatment with systemic
treatmentwealreadyachievedsignificantimprovementwithchanges blood pressure methylprednisolone 60
reduction of erythema and itching, absence of new skin120/70mmHg,heartrate80 mg/daywas initiated, later
lesions. The pregnancy ended in term with successful beats/min. The neurological gradually tapered to 20
childbirth.Noflareoftheskindiseasewasobservedinthe status was normal. The mg/day,togetherwithtopical
dermatological examination
puerperalperiod.
corticosteroids. Significant
found pathological skin
improvement was achieved
changesaffectingtheskinof
about the 10th day of the
Key word: Pemphigoid gestationis, herpes gestatio
theabdomen,theback,upper
nis, pregnancy, direct and indirect immunofluorescence, andlowerextremities,hands treatment with reduction of
erythema and itching and
methylprednisolone.
and feet. The eruption
absence of new skin lesions
consisted of erythematous,
(Fig. 5a, b). The pregnancy
INTRODUCTION:
urticarialike plaques with
Herpesgestationis(HG),currentlyknownaspemphi small tense vesicles upon endedintermwithsuccessful
childbirth. A healthy female
goid gestationis (PG), is a rare autoimmune blistering der them

and
newbornwithanthropometric
matosisofpregnancywithanincidencerangingupto1:50 multipleexcoriations. (Fig.
parameters within the nor
000pregnancies.[1]ThetermPGwasintroducedbyHolmes 1a, b) The mucous
mal ranges was born. No
andBlackin1982andislargelyusedinEuropebutinthe membranes and skin
flareoftheskindiseasewas
appendages were not
USAtheformernameHGisstillpreferred.[2,3]
observed in the mother in
affected. Routine laboratory
thepuerperalperiod.

552

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IMAB.2014,vol.20,issue6/

Fig. 2. Histopathology: A marked edema, with a

dense inflammatory infiltrate (predominantly with eosino
phils)inthedermis.

Fig. 3. Direct immunofluorescence (DIF) on peri

lesionalskin:lineardepositionofIgG(++)andC3(++)at
thecutaneousbasementmembranezone(BMZ).

Fig.4. Indirect immunofluorescence(IIF)onhuman

esophagussubstrate:circulatingIgGantiBMZantibodiesat
atiterof1:80.

Fig.1a,b. Confluent urticarialikeplaques covered

withtensevesiculesandexcoriationsontheabdomenand
backofthetrunk.

/JofIMAB.2014,vol.20,issue6/ http://www.journalimab-bg.org
553
glucocorticosteroids:reductionoferythemaandabsenceof
newskineruptions.

Fig. 5a, b. 10 daysafter treatment with systemic

DISCUSSION:
PGisaselflimiting,autoimmunesubepidermalbul
lousdermatosisofpregnancy.Itspathogenicmechanismre
mainsunclearbutisconsideredtoresultfromlackofrec
ognitionofthefetoplacentalunitbythematernalimmune
system.Thisleadstosubsequentproductionofantiplacen
tal antibodies. Similarly to bullous pemphigoid, the main
targetantigeninPGisBPantigenII(alsoknownasBP180),
in particular its noncollagenous 16A (NC16A) domain,
whichisconstituentofboththeplacentaandtheskin.The
skin pathologyinPG resultsfrom the abilityof the anti
placentalantibodiestocrossreactwiththesameproteinsof
the skin.Blister formation results from a complex
mechanism involving TH2lymphocytes, cytokines and
polymorphonuclearcells.[4,5,6]PGhasalsobeenreported
inassociationwithMHCclassIIHLAantigensDR3and
DR4.[1] The disease usually starts in the second or third
trimester of pregnancy and is characterized with an acute
onset of erythematous, urticarial papules and plaques that
progress to tense vesicles and blisters, followed bysevere
pruritus. The lesions usually arise on the abdomen, often
involvingtheumbilicus,andspreadcentrifugally.Recovery
occursgenerallyinafewweeksafterdeliverybutrelapses
arefrequentinsubsequentpregnancies.[7,8]Thediagnosis
of PG relies on theappropriate clinical presentation, the
histologicfindingsofasubepidermal blisteringandlinear
C3depositionalongtheBMZ,withorwithoutdepositionof
IgGonDIF.ELISABP180NC16Amaybeveryusefulin
such casesdue to its high sensitivity to detect circulating
554

autoantibodiesagainstNC16AdomainofBP180,whichis
themajorantigenicepitopeinPG.[9]
ThetreatmentofPGdependsontheseverityofthe
disease. Mild cases may be treated with topical cortico
steroids and oral antihistamines. Potent topical glucocor
ticoids and oral corticosteroids (prednisone 0.51 mg/kg/
day)arereservedformoreseriouscases.[1]Severalcases
resistanttocorticosteroidtherapyhavebeendescribed,but
successfully treated with intravenous immunoglobulin and
azathioprineorwithadjuvantimmunoadsorption.[10,11,12]
Fetalprognosisisgood,butearlyonsetin2ndtrimesterand
blister formation are risk factors for prematurity and low
birthweight.Rarelythenewbornmaybeaffectedbyvery
transitoryblisters.[7]
Inourcase,clinicalsuspicionofPGwasconfirmed
by DIF and IIF findings, as well as by ELISA
BP180NC16A, the latter beingstrongly positive. Systemic
treatment with methylprednisolone (0,5mg/kg/day) was
initiated during the pregnancy with gradual clinical
improvementuntildelivery.Ahealthyasymptomaticinfant
was born in term without cutaneous lesions. To date the
patienthasnotreportedaflare.

CONCLUSION
The presented case highlights the importance of a
timely clinical and immunohistological diagnosis of PG.
DifferentiationofPGfromotherconditionslikepruriticur
ticarial papules and plaques of pregnancy (PUPPP) is es
sentialbecausemanagementandoutcomesdiffer.Incasesin
whichtheclinicaldiagnosisisdifficult,immunofluores
http://www.journal- imab-bg.org
/JofIMAB.2014,vol.20,issue6/

cencetestsandELISAstudiesforantiBMZantibodiesare
useful in establishing the diagnosis. An interdisciplinary
approachisalsoofcrucialimportanceforthebenefitofthe
mother and child duringpregnancy, as well as during the
postpartumperiod.
Bonnetblan
c JM.
Chronic
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91,Gen.Vladimir
Vazovstr.,5800
Pleven,Bulgaria.
Email:
ivelina_yordanov
a@abv.bg,
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Addressforcorrespondence:
IvelinaYordanova,PhD.MD,
Assoc.Prof.
DepartmentofDermatologyand
Venereology,FacultyofMedicine,
MedicalUniversity,Pleven,