Pseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin,

AnBrasDermatol.2020;95(2):247---249

Anais

Brasileiros

de

Dermatologia

IMAGES

IN

DERMATOLOGY

Pseudoxanthoma

elasticum-like

papillary

dermal

elastolysis

in

non-exposed

skin

夽,夽夽

Nuria

Setó-Torrent

_,

_Maribel

_{Iglesias-Sancho}

_,

_Jorge

_{Arandes-Marcocci}

_,

María

Teresa

Fernández-Figueras

a_{DepartmentofDermatology,HospitalUniversitariSagratCor-GrupoQuirónSalud,Barcelona,Spain} b_{DepartmentofPathology,HospitalUniversitariSagratCor-GrupoQuirónSalud,Barcelona,Spain}

Received14April2019;accepted11August2019

Availableonline12February2020

KEYWORDS Dermis; Elastictissue; Female; Pseudoxanthoma elasticum

Abstract Pseudoxanthomaelasticum-like papillarydermalelastolysisisanacquiredelastic

tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic

involvement.Histopathologically,specialstainingofelasticfibersdemonstratesatotalor

par-tialband-likelossofelasticfibersinthepapillarydermis.Althoughultravioletradiationseems

tobeoneofthemainetiologicalfactorsinthisentity,wereportacaseofpseudoxanthoma

elasticum-likepapillarydermalelastolysisontheneckofawomanwhoworehijab.

©2020SociedadeBrasileira deDermatologia.PublishedbyElsevierEspa˜na,S.L.U.Thisisan

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

A 54-year-old female of Moroccan origin who habitually wears a hijab presented a 2 year history of mildly pru-ritic lesions on the neck. She denied systemic symptoms andfamily historyof similarfindings. Her medicalhistory included mixed anxiety-depressive disorder treated with olanzapine and sertraline. Physical examination revealed

夽 _{How to cite this article: Setó-Torrent N, Iglesias-Sancho}

M, Arandes-MarcocciJ, Fernández-Figueras MT.Pseudoxanthoma elasticum-likepapillarydermalelastolysisinnon-exposedskin.An BrasDermatol.2020;95:247---9.

夽夽_{StudyconductedattheHospitalUniversitariSagratCor-Grupo}

QuirónSalud,Barcelona,Spain.

∗_{Correspondingauthor.}

E-mail:nurisetorrent@gmail.com(N.Setó-Torrent).

white-to-yellowishmillimetricnon-follicularpapulesonthe lateral aspects of the neck and supraclavicular fossae (Fig.1).Dermoscopicexaminationshowedmultiple white-colorednon-follicularpapules,coalescingintoplaqueswith arboriformvessels(Fig.2).The biopsyshowedslight scle-rosisofthepapillarydermiswithneovascularizationanda mildinflammatoryinfiltrateincludinglymphocytesandsome melanophages(Fig.3).Inthesamearea,vanGiesonstain demonstratedadecreaseinthenumberofelasticfibersthat wereoften thin and fragmented (Fig.4) compatible with pseudoxanthomaelasticum-like papillary dermal elastoly-sis(PXE-PDE).Cardiac andophthalmologicalinvestigations performedwereunremarkable.

PXE-PDEisarareacquiredelastictissuedisorder charac-terizedbynon-follicular yellowishpapules coalescinginto plaqueswithpredilection for neck, supraclavicular fossae https://doi.org/10.1016/j.abd.2019.08.024

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

248 Setó-TorrentNetal.

Figure1 Whitishpapulesontheneckandsupraclavicular fos-sae.

Figure2 Multiplewhitishnon-follicularpapules,coalescing

intoplaqueswithlinearvessels,ondermoscopy.

andflexuralareas.1 _{Thelesionsareusuallyasymptomatic,}

butmilditchissometimesreported,asseeninourcase.To date, it affects exclusivelywomen mostly in middle age2

and it is not associated with any systemic involvement. Dermoscopic findings consist of multiple white-colored non-follicularpapules, coalescing intoplaqueswithlinear vessels.3

Histopathologically,hematoxylineosinstainingdoesnot revealanyspecificchanges.Thefocalinflammatorychanges present in our case have not been described previously; however,itispresumedthatelasticfiberlosscouldbethe resultofatransientphenomenonofinflammation.Special staining of elasticfibers withvan Gieson or orcein stains

Figure3 Slightsclerosisofthepapillarydermis,

neovascular-izationandamildinflammatoryinfiltrate(Hematoxylin&eosin,

x100).

Figure4 Reduction ofelasticfibers inthepapillarydermis

(vanGieson,x200).

are required to demonstrate a total or partial band-like lossof elasticfibersin thepapillarydermis.2 _{Calcification}

or fragmentation of the elasticfibers is absent. Immuno-histochemical studies usingmonoclonal antibodies against antibodyPcomponentcanalsodemonstratepartialor com-pletelossofelasticfibersinpapillarydermis.1_Thepresence

ofmelanophagesinthepapillarydermisconstitutesan addi-tionalhelpfuldiagnosticfeature.4

The cause of PXE-PDE remains unclear, and some etiopathogenic theories have been proposed: ultravio-letradiation, intrinsic aging,abnormalelastogenesis, and geneticorinheritablefactors.1,2_{Inourcase,ultraviolet}

radi-ation’setiopathogenictheoryisunlikelybecausethepatient worehijab.

Differentialdiagnosis ofPXE-PDEincludeswhitefibrous papulosisoftheneck,mid-dermalelastolysis,andpapillary dermalelastosis.Nevertheless, themaindifferential diag-nosis mustbeestablishedwithpseudoxanthomaelasticum (PXE),ahereditarydisordercausedbymutationonABCC6 gene. Clinically, PXE resembles PXE-PDE, but it appears at a youngerage, and itis usually associatedwithocular andcardiovascularcomplications. Histopathologically, PXE

Pseudoxanthomaelasticum-likepapillarydermalelastolysisinnon-exposedskin 249 presents fragmentation and calcification of elastic fibers

demonstratedwithvonKossastain.

TreatmentsforPXE-PDE,includingtopicalretinoids,have shownpoorresults2_{;however,non-ablativefractional}

resur-facing laser has demonstrated to be effective in some cases.5

HereinwepresentacaseofPXE-PDEinapatientwhodid notreceiveUVradiationbecausesheworehijab.Inour opin-ion,morestudiesareneededinordertobetterunderstand theetiopathogenesisofPXE-PDE.Itisimportantthat derma-tologistsrecognizethisentityanddifferentiateitfromPXE toavoidunnecessaryinvestigation.Clinicopathologic corre-lationisimportantandelastictissuestainsarerequiredto correctlydiagnosePXE-PDE.

Financial

support

Authors’

contributions

Nuria Setó Torrent: Approval of the final version of the manuscript; elaboration and writing of the manuscript; intellectualparticipationinthepropaedeuticand/or ther-apeuticconductofthestudiedcases;criticalreviewofthe literature;criticalreviewofthemanuscript.

Maribel IglesiasSancho:Approvalofthefinalversionof themanuscript;criticalreviewofthemanuscript.

JorgeArandesMarcocci:Approvalofthefinalversionof themanuscript;criticalreviewofthemanuscript.

MaríaTeresa FernándezFigueras: Approvalof the final versionofthemanuscript;criticalreviewofthemanuscript.

Conflicts

of

interest

Nonedeclared.

References

1.RevellesJM,MachanS,PielasinskiÚ,CamachoD,VallésL, San-tonjaC,etal.Pseudoxanthomaelasticum-likepapillarydermal elastolysis:immunohistochemicalstudyusingelasticfiber cross-reactivitywithanantibodyagainstamyloidPcomponent.AmJ Dermatopathol.2012;34:637---43.

2.PanagouE,RatynskaM,HeelanK.Pseudoxanthoma elasticum-like papillary dermalelastolysis: a casereport and reviewof literature.IntJDermatol.2019;58:93---7.

3.Ribeiro CP,Abuawad YG,Swiczar BCC,Valente NYS. Pseudox-anthoma elasticum-like papillary dermal elastolysis. An Bras Dermatol.2017;92:897---8.

4.RongiolettiF,IzakovicJ,RomanelliP,LanutiE,MitevaM. Pseu-doxanthomaelasticum-likepapillarydermalelastolysis:alarge caseserieswithclinicopathologicalcorrelation.JAmAcad Der-matol.2012;67:128---35.

5.FoeringK,TorbeckRL, FrankMP, SaediN.Treatmentof pseu-doxanthoma elasticum-like papillary dermal elastolysis with nonablativefractionalresurfacinglaserresultinginclinicaland histologicimprovementinelastinandcollagen.JCosmetLaser Ther.2018;20:382---4.