Exuberant case of erythema elevatum diutinum in a patient infected with HIV and hepatitis B virus,

AnBrasDermatol.2020;95(2):200---202

Anais

Brasileiros

de

Dermatologia

CASE

REPORT

Exuberant

case

of

erythema

elevatum

diutinum

in

a

patient

infected

with

HIV

and

hepatitis

B

virus

夽,夽夽

Sayuri

Aparecida

Hirayama

_,

_Cezar

_Arthur

_Tavares

_Pinheiro

_,

Isabelle

Maffei

Guarenti

_,

_Danise

_Senna

_Oliveira

a_{DepartmentofGeneralMedicine,TeachingHospital,UniversidadeFederaldePelotas,Pelotas,RS,Brazil}

b_{HIV/AIDSSpecializedCareService,UniversidadeFederaldePelotas,Pelotas,RS,Brazil}

c_{EmpresaBrasileiradeServic¸osHospitalares,TeachingHospital,UniversidadeFederaldePelotas,Pelotas,RS,Brazil}

Received2August2017;accepted27February2019 Availableonline12February2020

KEYWORDS Cutaneous; HepatitisBvirus; HIV; Leukocytoclastic; Vasculitis

Abstract Erythemaelevatumdiutinumisasmallvesselvasculitiswhichisbenign,rare,and chronic.Itisclinicallycharacterizedbyviolaceous,brown,oryellowishplaques,nodules,and papules.Ithasbeenassociated withautoimmune,infectious,andneoplasticprocesses.The followingcase describesapatientwithhepatitis Bvirusandhumanimmunodeficiencyvirus withCD4count<200mm3_{,HIV-seropositivefor16years,anddiagnosedwithhepatitisBvirusat}

thehospital.Thepatientwastreatedwithoraldapsone100mg/day,showingregressionafter sevenmonthsoftreatment.Theauthorsfoundthreecasesintheliteratureofassociationof erythemaelevatumdiutinum,humanimmunodeficiencyvirus,andhepatitisBvirus.

©2020SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.Thisisan openaccessarticleundertheCCBYlicense(http://creativecommons.org/licenses/by/4.0/).

Introduction

Erythema elevatum diutinum (EED) is a distinct form of cutaneousleukocytoclasticvasculitis,firstrecordedin1878

夽 _{Howtocitethisarticle:HirayamaSA,PinheiroCAT,GuarentiIM,} OliveiraDS. Exuberantcaseoferythemaelevatumdiutinumina patientinfectedwithHIVandhepatitisBvirus.AnBrasDermatol. 2020;95:200---2.

夽夽_{StudyconductedattheTeachingHospitaloftheUniversidade} FederaldePelotas,Pelotas,RS,Brazil.

∗_{Correspondingauthor.}

E-mail:sayuri.hirayama@hotmail.com(S.A.Hirayama).

by Hutchinson and later in 1879 by Bury. It was officially namedbyRadcliff-CrockerandWilliamsin18921_(apudJose

SK, 2016, p. 81). It occurs predominantly in adults from 40 to 60 years, being slightly more prevalent in men.2

Although its pathogenesis is still unknown, it is believed thatimmunecomplexesaredepositedonthewallofvenules andothervesselsbycontinuousantigenstimulationorother infections. Therefore, EED is associated with hematologi-cal,autoimmune,neoplastic,andinfectiousdiseases,such ashumanimmunodeficiencyvirus(HIV)infectionand hepati-tis.Thisdepositionofimmunecomplexesleadstoactivation ofthecomplementcascadeviaIL-8,withneutrophil chemo-taxis,releasinglysozymes,collagenases,myeloperoxidases,

https://doi.org/10.1016/j.abd.2019.02.013

0365-0596/©2020SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.ThisisanopenaccessarticleundertheCC BYlicense(http://creativecommons.org/licenses/by/4.0/).

Exuberantcaseoferythemaelevatumdiutinum 201

Figure1 Medialaspectofrightfootandlateralaspectofleft footwitherythematous-brownplaques.

andhydrolasesthatinducefibrindepositionandcholesterol crystalsinthecapillariesandvenules,leadingtodamage.1,2

Thisconditionischaracterizedbypapules,plaques,and nodules on the extensor surfaces of extremities, with a predilection for hands, feet, elbows, knees, and Achilles tendons,while sometimesbeingobservedonthefaceand ears.Theselesionsoccursymmetricallyandbilaterally;they areinitiallysoftanderythematousorpurpuric,with occa-sionalulceration.Overtime,lesionsmaydevelopwith hypo-orhyperchromiaintheeventofregression.Thenodularform israrer,usuallyoccurringinpatientswithHIVinfection. Pru-ritus andburning painin the lesionsmaybeobserved, as wellasarthralgias andocular alterations,suchasnodular scleritis, panuveitis, autoimmune keratolysis, and periph-eralkeratitis.1

Theauthorsdescribethecaseofapatientinfectedwith HIVandhepatitisBvirus(HBV)presenting with leukocyto-clasticvasculitisdiagnosedbybiopsyofskinlesions.

Case

report

This was a 43-year-old black male patient with a 16-year history of HIV infection who was using lamivudine+tenofovir+lopinavir/ritonavir (viral load 25,000copies/mL and CD4 count of 39cells/mm3_{). Three} yearsbefore,asingleand nodularlesionhad appearedin the right calcaneus; others lesions on the extensor face of the left lower limb and left elbow were observed in subsequentmonths.Alllesionswereitchyandprogressedin numberandsizeovertime.Physicalexaminationdisclosed symmetrically distributed erythematous-xanthochromic nodules onthe kneesand elbows, and linearplaques and erythematous-violaceous nodulesonthe ankles,toes, and plantar region (Figs. 1 and 2). The patient denied visual alterations and arthralgias. In this hospitalization, the patient wasdiagnosed with neurotoxoplasmosis, pneumo-cystosis,andhepatitisB(HbsAg,totalantiHBC,andHBEAg reagents with AST/TGO=20U/L and ALT/TGP=11U/L). Duetosuspicionoftuberousxanthoma,alipidprofilewas requested, which was normal. Kaposi’s sarcoma was also suspected.Biopsiesoftwoofthelesionswereperformed. The anatomopathological examination demonstrated neutrophilic dermatitis with marked leukocytoclasia and

Figure2 Brownishnodularlesionsontheknees.

Figure3 Anatomopathologicalexaminationoftheskinbiopsy demonstrating neutrophilic dermatitis, with marked leuko-cytoclasia and presence of fibrotic nodules surrounding the neutrophilic infiltrate, compatible with leukocytoclastic vas-culitis(Hematoxylin&eosin,×40).

Figure4 Lateral aspectofrightfoot aftersevenmonths of treatment,showinglesionsregression.

presence of fibrotic nodules surrounding the neutrophilic infiltrate (Fig. 3). Clinical and pathological correlation indicated EED. Treatment with dapsone 100mg/day was initiated, which resolvedthe lesionswithin sevenmonths (Fig.4).

202 HirayamaSAetal.

Discussion

The diagnosis of EED is clinical and histopathological; in the early stages, the latter presents leukocytoclastic vasculitiswithpolymorphonuclearcells,macrophages,and histiocytesinthedermis,andinthelatestage,granulation tissue, fibrosis, vascular proliferation, lymphohistiocytic inflammatory infiltrate, and focal areas containing neu-trophilswithleukocytoclasia. Inthelatestage,intra-and extracellular lipids (cholesterol deposits), although rare, may be observed. The differential diagnosis in the early phasemaybeSweet’ssyndrome,pyodermagangrenosum, facialgranuloma,drug-inducedrash,erythemamultiforme, cutaneousporphyria, and bullouspemphigoid. In the late stage,thedifferentialdiagnosisconsidersdermatofibroma, fibromatosis, necrobiotic xanthogranuloma, and tuberous xanthoma.InpatientswithHIVinfection,Kaposi’ssarcoma andbacillaryangiomatosisshouldalsobeconsidered.2

The first choiceof treatment is dapsone.3 _Alternatives

are colchicine, tetracyclines, niacinamide, and systemic corticosteroids such asprednisolone.4 _Topical

betametha-sone may also be used. New therapies with 5% topical dapsoneand plasmapheresishave been described.3 _Inthe

latestageofthedisease,thereislittleresponsetodapsone, given the predominant fibrosis. In this case, intralesional corticosteroids or lesion excision are the treatments of choice5_{Thediseasehasaprolongedduration,withreports}

ofspontaneousresolution rangingfromfivetotenyears.3

Relapsemayoccurafterdapsonediscontinuation.

In the literature, approximately 25 cases of EED and HIV infection have been described,6 _{one of which in}

Brazil,7 _{and threecases of HIV/HBV/EED co-occurrence.}8

EED is most commonly seen in patients with a CD4 count<200cells/mm3_{, and both immunosuppression and} antigen-antibody reactions caused by HIV and HBV are believed to be the triggering factors of this disease. Nonetheless,in the study by Muratoriet al.,9 _{in fourout}

offivepatientswithHIVinfection,thetriggeringfactorwas streptococcalinfection.Inthesepatients,thenodularform isthe mostprevalent,10 _{andthe palmoplantarregionmay}

beinvolved, asinthe present case. Differential diagnosis shouldincludebacillaryangiomatosis,Kaposi’ssarcoma,and rheumatoidnodules.1

Thepresent caseisrelevant,duetothefewreportsof EED/HIV/HBVpatientsintheliterature.Itiscommontofind skinlesionsinimmunosuppressedpatients,whichmay sug-gestdiseasesofvariousetiologies.Thus,histopathological confirmationisessentialtoestablishthediagnosis,thestage ofthedisease,andguidetreatment.

Financial

support

Nonedeclared.

Authors’

contributions

Sayuri Aparecida Hirayama: Approval of the final version of themanuscript; conception and planningof the study;

elaborationandwritingofthemanuscript;obtaining, ana-lyzing, and interpreting the data; critical review of the literature;criticalreviewofthemanuscript.

CezarArthur TavaresPinheiro: Preparation andwriting of themanuscript; effective participationinresearch ori-entation;intellectualparticipationinpropaedeuticand/or therapeuticconductofstudiedcases;criticalreviewofthe manuscript.

IsabelleMaffeiGuarenti:Approvalofthefinalversionof themanuscript;effectiveparticipationinresearch orienta-tion;criticalreviewofthemanuscript.

Danise Senna Oliveira: Intellectual participation in propaedeuticand/ortherapeuticconductofstudiedcases.

Conflicts

of

interest

Nonedeclared.

Acknowledgements

TheauthorswouldliketothankDr.IsabellaRoqueMiclos,a residentinClinicalMedicineattheUniversidadeFederalde Pelotas.

References

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