Case for diagnosis. Hyperpigmented and excoriated papules and nodules in a diabetic patient,

AnBrasDermatol.2020;95(6):757---759

Anais

Brasileiros

de

Dermatologia

WHAT

IS

YOUR

DIAGNOSIS?

Case

for

diagnosis.

Hyperpigmented

and

excoriated

papules

and

nodules

in

a

diabetic

patient

夽,夽夽

Catalina

Hasbún

_,

_Mauricio

_Sandoval

_,

_Sergio

_{González-Bombardiere}

a_{SchoolofMedicine,FacultyofMedicine,PontificiaUniversidadCatólicadeChile,Santiago,Chile}

b_{DepartmentofDermatology,FacultyofMedicine,PontificiaUniversidadCatólicadeChile,Santiago,Chile} c_{DepartmentofPathology,FacultyofMedicine,PontificiaUniversidadCatólicadeChile,Santiago,Chile}

Received10December2019;accepted4March2020 Availableonline13September2020

KEYWORDS Collagendiseases; Diabetesmellitus; Kidneyfailure, chronic; Paraneoplastic endocrinesyndromes

Abstract Reactiveperforatingcollagenosisisarareperforatingdermatosisclinically charac-terized by intenselypruritic hyperpigmentedpapules, plaques, and noduleswith a central keratoticplug.Histopathologyrevealstransepidermaleliminationofcollagenfibers.Its patho-physiology is still under investigation, but the acquired form has been linked to systemic conditions such asdiabetes mellitusandchronickidney disease. However, ithas also been describedasaparaneoplasticsyndrome.Theauthorspresentthecaseofa65-year-olddiabetic patientinwhichamyeloproliferativeneoplasmwassuspected.

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

Case

report

A65-year-olddiabeticfemalewithpoormetaboliccontrol (HbA1c14.9%)presentedwithatwo-monthhistoryof pru-riginouslesionsonthetrunkandextremities.

夽 _{How to cite this article: Hasbún C., Sandoval M,}

González-BombardiereS.Casefordiagnosis.Hyperpigmentedandexcoriated

papules and nodules in a diabetic patient. An Bras Dermatol.

2020;95:756---759.

夽夽_{ThisstudywasconductedattheDepartmentofDermatology,}

FacultyofMedicine,PontificiaUniversidadCatólicadeChile.

San-tiago,Chile.

∗_{Correspondingauthor.}

E-mail:masando1@uc.cl(M.Sandoval).

On physical examination, multiple umbilicated, hyper-pigmented papules with a central keratotic plug were observed(Figs.1and2),aswellasinguinalandcervical lym-phadenopathiesmeasuringupto2cmindiameter.Mucous membraneswereunaffected.

Laboratory tests revealed mild anemia with mild eosinophilia (hemoglobin 10.9g/dL, 940eosinophils/mL), elevated erythrocyte sedimentation rate (93mm/h), and elevatedlactatedehydrogenase(1000units/L).

What

is

your

diagnosis?

a) Prurigonodularis b) Lichenoiddrugeruption c) Lymphomatoidpapulosis d) Perforatingdermatosis

https://doi.org/10.1016/j.abd.2020.03.015

0365-0596/©2020PublishedbyElsevierEspa˜na,S.L.U.onbehalfofSociedadeBrasileiradeDermatologia.Thisisanopenaccessarticle

758 HasbúnCetal.

Figure 1 Clinical presentation of a 65-year-old female withgeneralizedumbilicated,hyperpigmented,andexcoriated papulesandnodules.

Figure2 Onmagnification,acentralkeratoticplugisvisible.

Skin biopsy showed a cup-shaped depression of the epidermis, with an overlying keratin plug containing col-lagen fibers, keratinousdebris,and inflammatory cells on H&E stained sections. Van Gieson staining demonstrated verticallyorientedcollagenfibersextrudingthroughthe epi-dermis(Figs.3and4).

Thepatientwastreatedwithantihistaminesand triamci-nolone.Asecondarystudyforamyeloproliferativeneoplasm was negative; she was referred to an endocrinologist to improvemetabolicmanagement.

Figure3 Photomicrographshowingacup-shapeddepression of the epidermis, with an overlying keratin plug contain-ingcollagenfibers, keratinousdebris, andinflammatorycells (Hematoxylin&eosin,_×100).

Figure4 Photomicrographshowingverticallyoriented colla-genfibersextrudingthroughtheepidermis(VanGieson,_×400).

Discussion

Reactiveperforatingcollagenosis(RPC)isararediseasein the spectrum of perforating dermatoses, showing epider-malperforationandtransepidermaleliminationofcollagen and/orelasticfibersashistologicfeatures.1

RPCmaybeclassifiedintohereditaryandacquiredforms. Thehereditarytypeappearsinearlychildhoodandis genet-ically determined by autosomal inheritance, whereas the acquiredform(ARPC)accompaniessystemicdiseases,most commonlydiabetesmellitus(DM)andchronickidneydisease (CKD).2_{However,ARPChasalsobeen associatedwithboth}

myeloproliferativeandsolidneoplasms.3

Clinically,thediseasepresentswitherythematousand/or hyperpigmented papules, plaques, and nodules. Lesions presentacentralumbilicatedorcrateriformhyperkeratotic plug,areintenselypruritic,andtheKoebnerphenomenonis observed.Afterhealing,atrophic,hypo-orhyperpigmented scarsarecommon.3,4_{Theselesionsappearinareasof}

super-ficialtraumaandaremostlikelyduetoitching.Indiabetic patients,vasculopathyofthedermishasbeenproposedasa synergisticfactor.5_{Thepalmoplantarregion,intertriginous}

areas,andmucousmembranesaregenerallyunaffected.3,6

RPCis a clinicaldiagnosis that requires histopathologi-calconfirmation, and itsfeaturesdepend on thestage of thedisease.Initially,degeneratecollagenfibersandnecrosis areseenextending intothereticulateddermis; epidermal hyperplasiamayalsobepresent.Inmoreadvancedlesions,

Hyperpigmentedandexcoriatedpapulesandnodulesinpatient 759 the epidermis develops a cup-shaped depression with an

overlyingbasophilickeratinplugconsistingofinflammatory cellsandkeratinousdebris.Verticalcollagenfibers,which stainredwithelasticVanGiesonstainingandbluewith Mas-son’strichromestaining,canbeobservedonthebaseofthe ulcerandextrudingthroughtheepidermis.7

Treatment goals are improvement of the pruritus and skin lesions and, most importantly, management of asso-ciatedinternaldiseases. Primarytherapybasedontopical corticosteroids,antihistamines,orantibioticshasbeen rec-ommended. In case of failure, second-line therapy with allopurinolshouldbeconsidered.8,9

Thiscaseemphasizestheneedtoconsideradiagnosisof ARPCwhenfacedwithchronicpruriticlesions,especiallyin thecontextofDMandCKD.However,eveninthisscenario, whenclinicalsuspicionforanassociatedneoplasmishigh, abasicstudyforinternalmalignanciesmustbeperformed.

Financial

support

Nonedeclared.

Authors’

contributions

Catalina Hasbún: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data;effective participationin research orientation;criticalreviewoftheliterature;criticalreview ofthemanuscript.

Mauricio Sandoval:Approval of thefinal version of the manuscript;designand planningofthe study;intellectual participation in propaedeutic and/or therapeutic conduct ofthestudiedcases;criticalreviewofthemanuscript.

Sergio González-Bombardiere: Approval of the final versionof the manuscript;collection, analysis, and inter-pretationofdata;intellectual participationin propaedeu-tic/therapeuticconductofthestudiedcases.

Conflicts

of

interest

Nonedeclared.

References

1.KimRH,KwaM,AdamsS,MeehanSA,SteinJA.Giantacquired reactive perforating collagenosis in a patient with diabetes mellitus and metastatic breast carcinoma. JAAD Case Rep. 2016;2:22---4.

2.KarpouzisA,GiatromanolakiA,SivridisE,KouskoukisC.Acquired reactive perforating collagenosis: currentstatus. JDermatol. 2010;37:585---92.

3.WagnerG,SachseMM.Acquiredreactiveperforatingdermatosis. JDtschDermatolGes.2013;11:723---9.

4.PattersonJW.Theperforating disorders.JAmAcadDermatol. 1984;10:561---81.

5.KawakamiT,SaitoR.Acquiredreactiveperforatingcollagenosis associatedwithdiabetesmellitus:eightcasesthatmeetFaver’s criteria.BrJDermatol.1999;140:521---4.

6.FaverIR,Daoud MS,SuWP.Acquired reactiveperforating col-lagenosis.Reportofsixcasesandreviewoftheliterature.JAm AcadDermatol.1994;30:575---80.

7.OrmerodE,AtwanA,IntzedyL,StoneN.Dermoscopyfeatures ofacquiredreactiveperforatingcollagenosis:acaseseries. Der-matolPractConcept.2018;8:303---5.

8.LukácsJ,SchliemannS,ElsnerP.Treatmentofacquiredreactive perforatingdermatosis-asystematicreview.JDtschDermatol Ges.2018;16:825---42.

9.Tilz H, Becker JC, Legat F,SchettiniAP, Inzinger M,Massone C.Allopurinolinthetreatmentofacquiredreactiveperforating collagenosis.AnBrasDermatol.2013;88:94---7.