AnBrasDermatol.2020;95(1):102---104
Anais
Brasileiros
de
Dermatologia
www.anaisdedermatologia.org.brWHAT
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:joana.calvao.silva@gmail.com(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.1Althoughinitially
reportedmainlyinJapanesemen,italsoaffectsCaucasian women,fromthe5thtothe9thdecadesoflife.2WFPNisa
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.3,6Despiteitsbenignnature,WFPN
maybecosmeticallyundesirableandsometimesitchy.Until now,noeffectivetreatmentshavebeenestablished.2Some
topicalagentsliketretinoinandantioxidantsweretried,but improvementwasnotnotable.2,7Surgicalexcisionmaybean
alternativechoiceoftreatmentincaseswithcircumscribed lesions.2Moreover,sinceWFPNisarguablyafeatureof
pho-toagingorintrinsicaging,fractionalnon-ablativelaserhas beenrecentlytriedwithgoodresults.2However,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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