AnBrasDermatol.2020;95(1):71---74
Anais
Brasileiros
de
Dermatologia
www.anaisdedermatologia.org.brCASE
REPORT
Eruptive
disseminated
Spitz
nevi
---
Case
report
夽,夽夽
Pablo
Vargas
a,∗,
Rodrigo
Cárdenas
b,
Roberto
Cullen
a,
Andrés
Figueroa
caDepartmentofDermatology,FacultyofMedicine,UniversityofChile,Santiago,Chile
bClínicaAlemanadeValdivia,Valdivia,Chile
cDermatologyService,UniversityofChileClinicalHospital,UniversityofChile,Santiago,Chile
Received26June2018;accepted14January2019 Availableonline12December2019
KEYWORDS
Epithelioidand spindlecell;
Immunohistochemistry; Neviandmelanomas; Nevus
Abstract Spitznevus isa benignmelanocytic lesion,which presentsin severalways: soli-tary,agminated,ordisseminated.Thedisseminatedvariantisuncommon;itmayhavearapid evolution(theeruptiveform)andbedifficulttomanage.Thisreportpresentsthecaseofa 24-year-old patientwithmultiplepapulesonhislimbs,which hadappearedfour years pre-viously.Onphysicalexamination,120pinkandskin-coloredpapuleswereseen,whichunder dermoscopywere observedtobehomogeneous,pinkvascularlesions. Histopathologicstudy revealedepithelioidcellsarrangedingroupsorsinglyinthedermisanddermo-epidermal junc-tion.TheywereHMB-45positiveinthesuperficialdermis,andKi-67<1%.Giventhesefindings, adiagnosisoferuptivedisseminatedSpitzneviwasmade.
©2019SociedadeBrasileira deDermatologia.PublishedbyElsevierEspa˜na,S.L.U.Thisisan openaccessarticleundertheCCBYlicense(http://creativecommons.org/licenses/by/4.0/).
Introduction
Spitznevusis abenignmelanocytic lesion,whichpresents inawidevarietyofclinicalandhistopathologicexpressions. Its biological behavior over time tends to be uncertain.
夽 Howtocitethisarticle:VargasP,CárdenasR,CullenR,Figueroa
A.EruptivedisseminatedSpitznevi---Casereport.AnBrasDermatol.
2020;95:71---4.
夽夽StudyconductedattheDepartmentofDermatology,Facultyof
Medicine,UniversityofChile,Santiago,Chile.
∗Correspondingauthor.
E-mail:[email protected](P.Vargas).
It is classified into solitary, agminated, and disseminated forms.DisseminatedSpitzneviis ararepresentation that may have a rapid (eruptive) evolution, or may develop over a prolonged period of time, several years (non-eruptive).1,2
Thisreportpresentsthe caseof aChileanpatientwith eruptiveSpitznevi,giventheunusualnatureofthisclinical variantandthefewreportsintheliterature.
Case
report
A 24-year-old male patient, with no significant morbid history. He was referred to this hospital because of the appearance,fouryearspreviously,ofmultipleraisedlesions https://doi.org/10.1016/j.abd.2019.01.010
0365-0596/©2019SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.ThisisanopenaccessarticleundertheCC
72 VargasPetal.
Figure1 (AandB)Pinkandskin-coloredpapules,withawarty appearanceandasessilebase.
onthe arms,elbows,thighs, andknees.They hadarapid progressiveevolutioninsizeandnumber,withnomention of pain, pruritus, hemorrhage, or other problems. Some lesions, mainly on the arms, had spontaneously resolved after ten to12 months. On physical examination approx-imately 120 pink and skin-colored papules were seen, with a warty appearance and a sessile base. Some were pedunculated. They were from 2 to 9mm in diameter, andwere symmetrically distributed onthe knees, thighs, elbows, and arms (Fig. 1). Dermoscopy showed homoge-neouspinkvascularlesions,withsmallscalesonthesurface (Fig.2).Excisionalbiopsywasperformedonthreelesions, whichrevealedessentiallysimilarfeatures:asymmetrical andwell-circumscribedcompoundmelanocyticproliferation composed of epithelioid cells with abundant eosinophilic cytoplasm arrangedin groups or singly in the dermis and dermo-epidermaljunction.Therewerevariableamountsof melanin pigment, hyalinization, and vascularization. Nei-thersignificantcytologicalatypianormitosiswereobserved (Fig.3).Immunohistochemicalstudy showedSOX10 witha
Figure 2 Dermoscopy: homogeneous pink vascular lesion, withsmallscalesonthesurface.
nuclearstainingpattern inmelanocytes, S100proteinwas positive,andtheKi-67labelingindexwas<1%(Fig.4).Given these findings, a diagnosis of eruptive disseminated Spitz neviwasmade,andthepatientagreedtothesurgical exci-sionofthemainlesionsundergeneralanesthesiaandstrict follow-up.
Discussion
Eruptive disseminatedSpitzneviwasdescribedin 1974as juvenileeruptivemelanoma,byWallaceetal.3Itisarare pathology withonly 27 reports in theliterature todate.4 The present case correlates with those described, given that the average age for the onset of the clinical
symp-Figure3 (AandB)Asymmetricalandwell-circumscribedcompoundmelanocyticproliferation,arrangedinepitheloidcellnests (A.Hematoxylin&eosin,×40,B.Hematoxylin&eosin,×100).(C)Epithelioidmelanocyteswithabundanteosinophiliccytoplasm, vesicularnucleus,andnucleolus(C.Hematoxylin&eosin,×400).
EruptivedisseminatedSpitznevi 73
Figure4 Immunohistochemicalstudy.(A)SOX10withanuclearstainingpatterninmelanocytes.(B)S100proteinwaspositive. (C)S100proteinwaspositive.
tomsis21years.Therearereportsfromnewbornpatientsto 35-year-old adults. The majority are women and in most cases there are no trigger factors. Isolated associations havebeendescribedwithchemotherapy,intravenousdrugs, surgery,exposuretothesun,andpregnancy.5,6Thelesions usually appear on the trunk and the proximal limbs. There have been four cases of spontaneous partial res-olution, a condition observed in the present patient. It is of utmost importance to perform differential diagno-sis, todistinguish between this and metastatic malignant melanoma,alsotakingintoaccountmultiplejuvenile xan-thogranuloma, urticaria pigmentosa, and dysplastic nevi syndrome.
In adults, a solitary Spitz nevus is usually treated by surgical removal,after clinical diagnosis, givenits uncer-tainbiological behavior. Inpractice,this is difficult todo inthe eruptive variant,considering thenumber oflesions seen. Various therapeutic options have been tried, such as electrocoagulation,7 liquid nitrogen,8 and imiquimod,9 without success. Although malignization has not been reported,thefewcasesreportedanditserraticevolution requires offering the patient several therapeutic options, fromconservativemanagementwithclinicalfollow-upand regular dermoscopy, to active management with topi-cal or surgical therapy, as is the case with the present patient.10
Financial
support
Nonedeclared.
Authors’
contribution
PabloVargas:Approvalofthefinalversionofthemanuscript; conception and planning of the study; elaboration and writing of the manuscript; obtaining, analyzing, and interpretingthedata;effectiveparticipationinresearch ori-entation;criticalreviewoftheliterature;criticalreviewof themanuscript.
Rodrigo Cárdenas: Approval of the final version of the manuscript;obtaining,analyzing,andinterpretingthedata; critical review of the literature; critical review of the manuscript.
Roberto Cullen: Approval of the final version of the manuscript;obtaining,analyzing,andinterpretingthedata; criticalreviewoftheliterature.
Andrés Figueroa: Approval of the final version of the manuscript;obtaining,analyzing,andinterpretingthedata; intellectualparticipationinpropaedeuticand/or therapeu-tic conduct of the cases studied; critical review of the manuscript.
Conflicts
of
interest
Nonedeclared.
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