Pseudo-wound infection after a caesarean section: Case report of unrecognized Pyoderma Gangrenosum

University of Groningen

Pseudo-wound infection after a caesarean section

van Donkelaar, Carlina E.; de Haan, Johanna M. H.; Lange, Johan F. M.; de Vries, Marjolijn;

Horvath, Barbara

Published in:

International journal of surgery case reports

DOI:

10.1016/j.ijscr.2020.03.041

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van Donkelaar, C. E., de Haan, J. M. H., Lange, J. F. M., de Vries, M., & Horvath, B. (2020).

Pseudo-wound infection after a caesarean section: Case report of unrecognized Pyoderma Gangrenosum.

International journal of surgery case reports, 69, 79-82. https://doi.org/10.1016/j.ijscr.2020.03.041

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ContentslistsavailableatScienceDirect

International

Journal

of

Surgery

Case

Reports

Pseudo-wound

infection

after

a

caesarean

section:

Case

report

of

unrecognized

Pyoderma

Gangrenosum

Carlina

E.

van

Donkelaar

_,

_Johanna

_M.H.

_de

_Haan

_,

_Johan

_F.M.

_Lange

_,

Marjolijn

de

Vries

_,

_Barbara

_Horváth

a_{DepartmentofObstetricsandGynecology,ZiekenhuisgroepTwente,Almelo,theNetherlands} b_{DepartmentofSurgery,UniversityMedicalCenterGroningen,Groningen,theNetherlands} c_{DepartmentofDermatology,UniversityMedicalCenterGroningen,Groningen,theNetherlands}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received19February2020

Receivedinrevisedform15March2020 Accepted19March2020

Availableonline13April2020

Keywords:

Pyodermagangrenosum Abdominalnecrosis Caesareansection

a

b

s

t

r

a

c

t

BACKGROUND:Pyoderma Gangrenosum(PG) is arareauto-inflammatory disease, characterizedby

painfululcerativeskin-lesionsoftendevelopingatsitesofinjuryorsurgerybecauseofthetypicalpathergy

phenomena.WedescribeanunusualcaseofPGafteracaesareansectionwithexcessiveextra-cutaneous

manifestationwithininternalorgans.

PRESENTATIONOFCASE:A21-year-oldDutchprimigravidadevelopedsignsofsepsisafteracaesarean

sec-tion.Despiteantibiotictreatment,fastclinicaldeteriorationoccurred.Explorationofthewoundshowed

necrosisoftheuterusandsurroundingtissues.Duetotheprogressionofnecrosis,consecutive

debride-mentprocedureswereexecutedresultinginasubstantialabdominalwalldefect.Theprogressiveclinical

courseofthenecrosiscombinedwithabsenceofpositivewoundculturesandhistologyofprominent

interstitialneutrophilicinfiltration,ledtothediagnosis‘PyodermaGangrenosum’.Treatmentwithhigh

dosecorticosteroidsledtorapidregressionofthedisease.Afterseveralweeks,theabdominalwalldefect

wassurgicallycorrectedundersystemiccorticosteroidtherapy.

DISCUSSION:ThiscaseofPGisuniqueduetotheexcessiveextra-cutaneouspresentation,which

con-tributedtodelayeddiagnosis.Severalsurgicalinterventionsintheactivestageofdiseaseresultedin

expansionofPGandsubstantialmorbidityforthepatient.

CONCLUSION:Post-operativePGcanmimicinfectiousdiseases,buttreatmentissubstantiallydifferent.

ThiscaseofextensivePGhighlightstheimportanceoftimelyrecognitionandtreatmentofthedisease

toreduceiatrogenicmorbidity.

©2020TheAuthor(s).PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.Thisisanopen

accessarticleundertheCCBYlicense(http://creativecommons.org/licenses/by/4.0/).

1. Introduction

PyodermaGangrenosum(PG)isarareneutrophilicdermatosis, closelyrelatedtoauto-inflammatorydiseases.Theincidencerate isapproximately3–10patientspermillionperyear,withapeak incidencebetween20and50yearsandwomenmorecommonly affected[1,2].

Typically,PGlesionsdevelopatsitesofinjurycausedbytrauma orsurgery,alsoknownasthepathergy phenomena.Duetothe highinflammatory load,PGis oftenmistakenfor severe bacte-rialinfection,suchasnecrotizingfasciitis.Limitedknowledgeof

Abbreviations: C-section,caesareansection;NPWT,negativepressurewound therapy;PG,PyodermaGangrenosum.

∗ Correspondingauthorat:ZiekenhuisgroepTwente,DepartmentofObstetrics andGynecology,Zilvermeeuw1,7609PPAlmelo,theNetherlands.

E-mailaddress:c.e.van.donkelaar@umcg.nl(C.E.vanDonkelaar).

1 _{Bothauthorscontributedequally.}

PGamongstphysicians,otherthandermatologists,contributesto delayeddiagnosisandtreatment.

WedescribeanatypicalcaseofPGafteracaesareansection (C-section)withextra-cutaneousinvolvementaffectingtheinternal organs,inwhichdelayeddiagnosisattributedtoiatrogenic dam-ageandahighmorbidityofthedisease.Thiscase-reporthasbeen reportedinlinewiththeSCAREcriteria[3].

2. Case

The21-year old patientunderwent anuncomplicated emer-gencyC-sectionat32+1weeksduetopretermlaborandbreech position.Thepregnancywasuncomplicatedsofar,andher medi-calhistoryreportednorelevantinformation.Twodayspostpartum, shedevelopedfever(39.5 degreesCelsius)incombination with increasedCRPof236(normalvalue<10)andleukocytosis(35.9 109_{/L,normalvalue4–10×10}9_{/L).Physicalexaminationrevealed}

paininthelowerabdominalregionwithanenlargeduterus. Ultra-soundexcludedretainedplacentalfragment.Antibiotictreatment

https://doi.org/10.1016/j.ijscr.2020.03.041

2210-2612/©2020TheAuthor(s).PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.ThisisanopenaccessarticleundertheCCBYlicense(http://creativecommons. org/licenses/by/4.0/).

80 C.E.vanDonkelaaretal./InternationalJournalofSurgeryCaseReports69(2020)79–82

Fig.1. A:TheC-sectionwoundwithpustularedgesandviolaceousborders(7th daypostpartum).B:Theabdominalwounddehiscenceaftermultipledebridement’s (19thdaypostpartum).Woundedgesareviolaceouswithapustularbullaonthe lowerrightabdomen,surroundedbydiffuseerythema.

wasstarted under the diagnosis of endometritis. Multiple

cul-tures(blood,woundandvaginal)remainednegative.On5thday

postpartum,theC-sectionwoundshowedpurulentdischargeand

wounddehiscence. Partial openingof thewound confirmedan

intactabdominalfascia(Fig.1a).Woundtherapywasstartedand

antibiotictreatmentcontinued.

Lack of clinical improvement necessitated a CT-scan which revealeddiscolorationoftheanterioruteruswall,suspectfor necro-sis.Soonafterwards,thepatientdevelopedsystemicinflammatory responsesyndromewithacutekidneyfailure.Treatmentwas initi-atedaccordingtothesepsisprotocolandshewasadmittedtothe intensivecareunit(ICU).Exploratory laparotomyshowed puru-lentnecrotic tissueontheloweruterinesegmentoftheuterus (betweentheuterusandbladder),alongtheabdominalmuscles, andtheretroperitonealtissuesurroundingtheureters.Extensive debridementwasperformed.Duetonecrosisprogressiontwo addi-tionallaparotomieswithdebridement wereperformed.Still,all bloodandwoundculturesremainedfreeofmeaningfulpathogens. Additional serology blood tests ruled out human immunodefi-ciencyvirus,syphilisandsystemiclupuserythematosus.On19th daypost-partum,theabdominalwoundedgesonceagainshowed dehiscencewithsignsofnecrosis(Fig.1b).Afterthethird debride-ment, a negative pressure wound therapy (NPWT) systemwas appliedtocovertheabdominalwalldefect.Additionally,two para-colicdrainswereplacedintra-abdominally.

Thecomplexityofthecaseandprogressionofillnessrequired transfer to an academic hospital (20 days postpartum). Explo-rative laparotomy showed a substantial abdominal wall defect withnecroticwoundedgeswhichweredebrided.Anew NPWT-systemwasplacedonthewound.Thedrainentrywoundsshowed nosignsofinflammation(Fig.2a).However,twodayslater,the drainentrywoundsshowednewlyformedpurulentulcers(Fig.2b), whichwerenotrelatedtothenecrotictissuearoundthe abdomi-nalwound.Adermatologistwasconsultedandbiopsiesweretaken, showingprominentinterstitialneutrophilicinfiltrationwitha

dif-Fig.2.A:TheabdominalwoundwithNPWT-systeminsitu(20thdaypostpartum). B:Newulcerationaroundtheleftandrightparacolicdrainsisvisible(22ndday postpartum).

ferential diagnosis of infection, Sweet’s syndrome and PG. The combinationoftheclinicalcourse,progressionundersurgicaland antibiotictreatmentandabsenceofmeaningfulpathogensinthe cultures,ledtothediagnosisofPG.

Immediatetreatmentwithhigh-dosesystemiccorticosteroids (Prednisolone1mg/kg)ledtorapidclinicalimprovement. Treat-mentwaslaterexpandedwithaT-cellinhibitor(Cyclosporine-A 5mg/kg), and the corticosteroid dose reduced. Twelve weeks’ post-partum, the patient underwent abdominoplasty (Fig. 3) underimmunosuppressivetreatment.Immunosuppressive treat-ment wastapered off over several monthsand hassince been stopped.Severalfollow-upshaveshowednosignsofre-activation ofPG.

3. Discussion

TheexactunderlyingpathophysiologyofPGisunknown,but involvesdysregulationoftheimmuneresponse.Patientshave sig-nificantoverexpressionofcytokinesandchemokines,andthereis evidenceofgenemutationsinseveralauto-inflammatorygenes [1].Morethan50%ofthepatientshaveanassociatedsystemic

dis-Fig.3.Theabdomenafterabdominoplasty,3monthspostpartum.

ease,mostcommonlyinflammatoryboweldisease(Crohn’sDisease

andColitisUlcerosa).Otherrelateddiseasesarearthritisand

spe-cifichematologicalmalignancies[1,2].Inthispatient,athorough

work-upforassociatedconditionsremainednegative.

Although diagnostic criteria have been proposed by Maver-akisetal.[2],PGremainsdifficulttodiagnose.Thetypicalskin lesionofthissubtypeisapainfululceratingwoundwitha viola-ceous,elevated/underminedborder,oftenprogressivelyspreading peripherally.The lesionsareaccompanied byspikingfever and general malaise and laboratory findings include high CRP and leukocytosis[1,4].

PGdevelopsin25–50%ofthecasesaftersurgeryortraumatothe skinduetoneutrophilactivation,theso-calledpathergyphenomen [3,4].PGisoftendescribedafterbowelsurgery(mostcommonly locatedparastomal)and breastsurgery [1,5], butrarelyafter C-sections.Interestingly,manyofthecasesdescribedinliterature, reportaprematureC-sectionduetopretermlabororsuspected fetaldistress[6–10].AnassociationwithPGandprematuredelivery hasnotyetbeenestablished.

PGaftersurgeryisoftenmistakenforabacterialwoundinfection withsubsequentdelayinappropriatetreatment.Inacase-seriesof 36PGpatients,29patientsweremisdiagnosedaswoundinfection and13patientsreceiveddebridementofthelesions[11]. Remark-ableinthiscase,isthatthePGappearedlargelyintra-abdominal insteadofintra-cutaneous, whichincreasedthesuspicion ofan infectiousdiseasesuchasnecrotizingfasciitis,andfurtherdelayed diagnosis.Extra-cutaneousmanifestation ofPGis rare,a recent reviewreported96casesdescribedbetween1973and2018[12].Of thesecases,pulmonarymanifestationwasthemostcommon,but genitalandcervicalmanifestationsofPGhavealsobeenreported. Therehavebeennopreviousreportssuggestinguterine manifes-tationof PG,sothis case maybe thefirst,although it remains difficulttodetermineifthePGwasactuallyinitiatedintra-uterine, orwhetheritexpandedfromtheskinintotheinternalorgans.

TreatmentofPGisbasedonlocalorsystemicimmune suppres-sion,dependingontheclinicalcourse[1].Forsystemictreatment, high-doseprednisone is thepreferredchoice,withrapideffect. Surgerywithactive PGmustbeavoided asaggravates the sur-roundingtissueandleadstoPGprogression[1].Inthiscase,the extensiveabdominaldebridement’sduringtheactivephaseofthe diseasemightcontributedtothedestructivecourse.

Afterthediagnosis,theNPWT-systemwassuccessfullyusedto bridgetheperiodofactivePGandafterthePGwasstabilized,the abdomencouldbeclosed.NPWTunderimmunesuppressionisno routinetreatmentofPGwounds,butafewdescribedcasespresent successfuluseintreatmentofdeeptissuewoundsrelatedtoPG [13].NPWTisknownforincreasingtissueperfusion,enhancing cel-lularproliferation,andreducingbacterialload[14].Furthermore, incaseofPGitseemstoresultinsignificantpainreduction[13].

Long-termoutcomeofpatientswithPGremainsunpredictable, even after effective treatment. Recurrence rates upto 70% are described, but are based on small numbers of patients [4]. In patientswithahistoryofPG,prophylacticadministrationof peri-operativeimmunosuppressivetherapyisrecommendedincaseof futuresurgicalprocedures.

Despiteadvancesindiagnosticsandtreatment,PGisstill asso-ciatedwithhighmorbidityand potentialmortality.Inthiscase, thedelayeddiagnosiscertainlycontributed tothehigh morbid-ity.Thispatientunderwentmanylaparotomiesatyoungage,with largeconsequences:theabdominalmuscleswerelargelyaffected andheruteruswasextirpated.Furthermore,becauseofthelong stayontheICUandsurgicalward,shewasnotabletotakecareof hernewbornandadequatelybond.Luckily,sherecoveredrelatively wellandupontodaynorecurrenceofthediseaseoccurred.

4. Conclusion

Pyodermagangrenosumisarareneutrophilicdermatosisthat can occur after surgery because of the pathergy phenomena. The clinicalsymptoms canmimic a secondary bacterialwound infection,consequently,thediseaseis oftennotrecognizedand mistreated.Timelyrecognitionandadequatetreatmentofthe dis-easeisofutmostimportancetoavoidiatrogenicmorbidity.

DeclarationofCompetingInterest

Noconflictofinterestistodeclare.

Sourcesoffunding

FundingfromtheUniversityofGroningen,theNetherlands,was receivedforpublicationofthisarticle.

Ethicalapproval

Ourlocalinstitutionalboarddecidedthatnoethicalapprovalis necessaryforthiscase-report.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.

Authorcontribution

CEDandJMHHdraftedthemanuscript.

JFM,MVandBHwereinvolvedintreatmentofthepatientand reviewedthemanuscript.

Allauthorsreadandapprovedthefinalmanuscript.

Registrationofresearchstudies

Nameoftheregistry:Notapplicable.

UniqueidentifyingnumberorregistrationID:NA.

Hyperlinktoyourspecificregistration(mustbepublicly acces-sibleandwillbechecked):NA.

Guarantor

CEvanDonkelaaristheguarantor.

Provenanceandpeerreview

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