Multiple whitish papules on the posterior neck of an elderly woman,

AnBrasDermatol.2020;95(1):102---104

Anais

Brasileiros

de

Dermatologia

WHAT

IS

YOUR

DIAGNOSIS?

Multiple

whitish

papules

on

the

posterior

neck

of

an

elderly

woman

夽,夽夽

Joana

Calvão

,

Bárbara

Roque

Ferreira

,

José

Carlos

Cardoso

DepartmentofDermatovenereology,CentroHospitalareUniversitáriodeCoimbra,Coimbra,Portugal

Received18February2019;accepted19May2019

Availableonline18December2019

KEYWORDS

Elastictissue; Neck;

Pseudoxanthoma elasticum

Abstract White fibrous papulosis of the neck is a rare entity, with fewer than 50 cases

described.Itisabenignpathologywhosemaininterestliesinitsbroaddifferentialdiagnosis,

especiallywithpseudoxanthomaelasticum.Theauthorsreportthecaseofa77-year-oldwoman

withmultipleyellow-whitemonomorphicpapulesontheposteriorcervicalregion,withyears

ofevolution.Cutaneousbiopsyrevealedanodularareainthesuperficialandmiddlereticular

dermis,withslightthickeningofthecollagenfibersandfocallyenlargedelasticfibers,aspects

highlightedintheVerhoeffstainingthatadditionallyshowedabsenceofelasticfibers inthe

papillarydermis.

©2020SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.Thisisan

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

Case

report

A77-year-oldwoman,whowasotherwisehealthy,presented with multiple white-yellowish millimetric, monomorphic, non-follicular papules, on the posterior cervical region (Figs.1and2).Theselesionshadbeenpresentforseveral years, and except for mild pruritus, were asymptomatic.

夽 _{Howtocitethisarticle:CalvãoJ,FerreiraBR,CardosoJC.}

Mul-tiplewhitishpapulesontheposteriorneckofanelderlywoman.An BrasDermatol.2020;95:102---4.

夽夽_{StudyconductedattheDepartmentofDermatovenereology,}

CentroHospitalareUniversitáriodeCoimbra,Coimbra,Portugal.

∗_{Correspondingauthor.}

E-mail:[email protected](J.Calvão).

Physicalexamination, includingperipheralpulses and car-diovascularevaluation,revealednormalfindings.Cutaneous biopsyrevealedanill-definednodularareainthesuperficial andmid-reticulardermis,characterizedbyslightthickening ofthecollagenfibersandbyfocallyenlargedelasticfibers (Fig.3).ThesefindingswerehighlightedintheVerhoeff-Van Gieson staining, which further showed absence of elastic fibersinthepapillarydermis(Fig.4).Calcificationofelastic tissuewasnotseen.

Discussion

Clinicopathological correlation favored the hypothesis of white fibrous papulosis of the neck (WFPN). In this context,theauthorsdecidedonawatch-and-waitapproach; thelesionscurrentlyremainstable.

https://doi.org/10.1016/j.abd.2019.05.003

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

Multiplewhitishpapulesontheposteriorneckofanelderlywoman 103

Figure1 Multiplesmallwhitishpapulesontheposterior

cer-vicalregion.

Figure2 Multiplesmallwhitishpapulesontheposterior

cer-vicalregion.

Figure3 Mildsolarelastosisandthickeningofcollagen

bun-dlesinthesuperficialandmid-reticulardermis(Hematoxylin&

eosin,×40).

WFPN was first described by Shimizu et al. in 1985. A few years later, the same authors reported this condi-tioninagroupof32Japanese patients.1_{Althoughinitially}

reportedmainlyinJapanesemen,italsoaffectsCaucasian women,fromthe5thtothe9thdecadesoflife.2_WFPNisa

rareacquiredfibroelastolyticdisorder,whichpresents clin-icallyasyellowish-whiteroundor ovalpapules that occur morefrequentlyontheposterior neckregion,butalsoon the back, without associatedsystemic manifestations. Its etiopathogenesis,notfullyunderstood,seemstoberelated tointrinsicskinphotoaging,butisprobablymultifactorial.3,4

Figure 4 Thickening of collagen bundles and multifocal

coarsethickeningoftheelasticfibers(Verhoeff,×200).

Theclinicalrelevanceofthisentityliesinitsbroad differ-entialdiagnosis,especiallywithpseudoxanthomaelasticum (PXE),ageneticdiseasecausedbyamutationintheABCC6 (ATP-bindingcassettesub-familyCmember6)geneandis associatedwithectopicmineralizationoftheskin,eyes,and bloodvessels.5,6 _{However,in contrasttothe latter, WFPN}

usuallyappearslateinlife,isnotassociatedwithsystemic complications,anddoesnotrequirefurtherinvestigation.3

Histologicalexamination revealsdermal fibrosis consisting ofcircumscribedareasofthickenedcollagenbundlesinthe papillaryandmid-reticulardermis,aswellasavariableloss ofdermalelastictissue.3,6_{Despiteitsbenignnature,WFPN}

maybecosmeticallyundesirableandsometimesitchy.Until now,noeffectivetreatmentshavebeenestablished.2_Some

topicalagentsliketretinoinandantioxidantsweretried,but improvementwasnotnotable.2,7_{Surgicalexcisionmaybean}

alternativechoiceoftreatmentincaseswithcircumscribed lesions.2_{Moreover,sinceWFPNisarguablyafeatureof}

pho-toagingorintrinsicaging,fractionalnon-ablativelaserhas beenrecentlytriedwithgoodresults.2_{However,noneofthe}

treatmentspresentsufficientevidenceintheliterature.

Financial

support

Nonedeclared.

Authors’

contribution

Joana Calvão: Approval of the final version of the manuscript; composition of the manuscript; collection, analysis, and interpretation of data; participation in the design of the study; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases;critical review of the literature; critical review of themanuscript.

BárbaraRoqueFerreira:Approvalofthefinalversionof themanuscript;collection,analysis,and interpretationof data;participationinthedesignofthestudy;criticalreview ofthemanuscript.

JoséCarlosCardoso:Approvalofthefinalversionofthe manuscript;conceptionand planningof thestudy; collec-tion,analysis,andinterpretationofdata;criticalreviewof themanuscript.

104 CalvãoJetal.

Conflicts

of

interest

Nonedeclared.

References

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