Granulomatous pigmented purpuric dermatosis: report of a Latin-American case with blaschkoid distribution

AnBrasDermatol.2019;94(5):582---585

Anais

Brasileiros

de

Dermatologia

CASE

REPORT

Granulomatous

pigmented

purpuric

dermatosis:

report

of

a

Latin-American

case

with

blaschkoid

distribution

夽,夽夽

Daniela

Carvajal

_,

_Claudia

_Quiroz

_,

_Claudia

_Morales

_,

_Javier

_Fernández

a_{DermatologyDepartment,FacultyofMedicine,UniversityofChile,Santiago,Chile} b_{DermatologyUnit,ClinicalHospitalUniversityofChile,Santiago,Chile}

c_{PathologyUnit,ClinicalHospitalUniversityofChile,Santiago,Chile} d_{DermatologyUnit,HospitalSanJosé,Santiago,Chile}

Received6March2018;accepted4June2018

KEYWORDS

Dyslipidemias; Granuloma; Skindiseases, vascular

Abstract Granulomatous pigmented purpuric dermatosis clinically manifests as

hyperpig-mentedmaculaeandpetechiae,predominantlyonthelowerextremities.Histopathologically,it

ischaracterizedbyalymphocyticinfiltrateintheupperdermis,extravasatederythrocytes,and

hemosiderindeposits.Thereisaninfrequentvariantcalledgranulomatouspigmentedpurpuric

dermatosis,whichhistologicallyischaracterizedbythepresenceofnon-necrotizinggranulomas

associatedwiththeclassicfindingsofotherpigmentedpurpuricdermatoses.Itmorefrequently

affectsmiddle-agedwomenofAsianorigin,andpredominantlyonthelowerextremities.The

authorspresentthecaseofafemalepatientwithgranulomatouspigmentedpurpuricdermatosis

onthelowerextremitieswithblaschkoiddistribution.

©2019SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.Thisisan

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

夽 _{Pleasecitethisarticleas:CarvajalD,QuirozC,MoralesC,}

Fer-nándezJ.Granulomatouspigmentedpurpuricdermatosis:reportof aLatin-Americancasewithblaschkoiddistribution.AnBras Derma-tol.2019;94:582---5.

夽夽_{StudyconductedattheClinicalHospitalUniversityofChile,}

Santiago,Chile.

∗_{Correspondingauthor.}

E-mail:fernandez.moraga.javier@gmail.com(J.Fernández).

Introduction

Pigmented purpuricdermatosis(PPD)or capillaritis repre-sents a heterogeneous group of dermatoses of uncertain etiology, which are characterized by pigmented red to brown maculae and petechiae, predominantly on the lowerextremities.Classically, fiveclinical variantsof PPD have been described: purpura annularistelangiectodes of Majocchi, progressive pigmentary dermatosis of Scham-berg,pigmentedpurpuricdermatitisofGougerotandBlum, eczematoid-likepurpuraofDoucasandKapentanakes,and

https://doi.org/10.1016/j.abd.2019.09.002

0365-0596/©2019SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.ThisisanopenaccessarticleundertheCC BYlicense(http://creativecommons.org/licenses/by/4.0/).

Granulomatouspigmentedpurpuricdermatosis 583

Figure1 (A)Patientwithred-brownpatchesandpetechiaesymmetricallydistributedonthelowerextremities.(BandC)Lesions

ontheposterioraspectoftheleftleginalineardisposition,withblaschkoiddistribution.

lichen aureus.1 _{The classic histological findings include a}

superficial perivascularlymphocytic infiltrate,erythrocyte extravasation,andhemosiderindeposit,withoutvasculitis. AgranulomatousvariantofPPDhasbeenrecentlydescribed, whose main histological characteristic is the presence of non-necrotizing granulomassuperimposedwiththe classic findingsofPPD.2

This reportdetailsthecaseofafemaleLatin-American patientwithhyperpigmentedpatchesonthelower extremi-ties,someofthemwithablaschkoiddistribution,whoseskin biopsyrevealedthepresenceofnon-necrotizinggranulomas associatedwiththecommonfeaturesofPPD.

Case

report

A 62-year-old woman presented with a six-month history of asymptomatic pigmented patches on both legs. She also had a history of diabetes mellitus II, hypertension, dyslipidemia, and hypothyroidism, under treatment with metformin,losartan,atenolol,atorvastatin,and levothyrox-ine.Therewerenootherassociatedsymptomsor changes intheusualmedication.

Physical examination showed small red-brown patches of 2---3mm andnon-palpable petechiaesymmetrically dis-tributedonthelowerextremities(Fig.1A).Intheposterior aspect ofthe leftleg,thelesionshad alineardisposition withablaschkoiddistribution(Fig.1BandC).Dermatoscopy showedred-brownspotsandglobulesarrangedona back-groundofcoppery-redpigmentation(Fig.2).

Skinbiopsyshowedathinnedepidermis,lichenoid infil-trate in bands with lymphocytes in the papillary and reticular dermis (Fig. 3A), small non-necrotizing granulo-masin the dermis formed by deposits of epithelioidcells and multinucleated giant cells, surrounded by lympho-plasmacytic cells, without necrosis (Fig. 3B). Erythrocyte extravasationandafeweosinophilswereobserved,withthe absence of vasculitis. Hemosiderin deposits were demon-strated using Prussian blue iron stain (Fig. 3C). Periodic acid---Schiff(PAS)andZiehl---Neelsenstainingwerenegative.

Figure 2 Dermatoscopy of the lesions showed red-brown

spotsand globulesarrangedon abackgroundofcoppery-red

pigmentation.

Laboratorystudiesshowedelevatedlow-density lipopro-tein cholesterol and triglycerides. C-reactive protein and erythrocytesedimentationratewerenormal,withoutorgan dysfunction. The clinical history and histological findings confirmedthediagnosisofgranulomatousPPD.

Discussion

Granulomatous PPD was first described by Saito and Matsuoka in 1996, representing the last variety of PPD published.2 _{To date, only 27 cases of granulomatous PPD}

havebeenpublished,withaclearpredominanceinfemales (74%),between9and75yearsofage,andwithhalfofthe casesinAsianpatients.3_{Inrecentyears,reportsof}

granu-lomatousPPDhaveincreasedinCaucasianpatients,withno casespublishedamongtheLatin-Americanpopulation.

Clinically, granulomatous PPD presents as red-brown patchesandpetechiae,similartootherformsofPPD,with the lowerextremities being the most frequent location.4

584 CarvajalDetal.

Figure3 (A)Thinnedepidermis,lichenoidinfiltratewithlymphocytesandhistiocytesformingnon-necrotizinggranulomasinthe

dermisanderythrocyteextravasation(hematoxylin&eosin,×100).(B)Smallnon-necrotizinggranulomasinthedermisformed

bydeposits ofepithelioid cellsandmultinucleated giantcells(hematoxylin& eosin,_×400). (C)Erythrocyte extravasationand

hemosiderindepositsinthedermis,especiallyundertheinflammatorydepositswithgranulomas(Prussianblueironstain,×100).

The dermatoscopic findings in the present case are sim-ilar to other reports, describing multiple round-to-oval brown-red dots, globules, and patches in a background of non-networkedcoppery-red pigmentation.5 _{There have}

beenreportsoflinearunilateralcapillaritis,lichenaureus, and Schamberg disease with lesions arranged in a linear distribution.2,6 _{However, there are no reports to date of}

granulomatousPPDwithalinearorblaschkoiddistribution. Histologically, granulomatous PPD presents the classic findings of other forms of PPD, with the addition of the presence of lymphohistiocytic infiltrate and the forma-tionofnon-necrotizinggranulomasinthepapillarydermis, somearrangedaroundthevascularplexus.2,3_{Otherfindings}

includevacuolarchangesorlichenoidinfiltrateinthe der-moepidermal junction.4,7 _{The histopathologic differential}

diagnosisincludesotherdiseases thatpresent granulomas; infectiousdiseasessuchastuberculosisoratypical mycobac-teria,andnon-infectiousdiseasesuch assarcoidosis,drug reaction, metastatic Crohn’s disease, granulomatous vas-culitis,ormycosisfungoides.5

TheetiologyofgranulomatousPPDisstilluncertain.This variant has been associated with hyperlipidemia in more than50%ofthecases.3_{Ithasbeenpostulatedthat}

hyperlipi-demiacouldcausechronicinflammationandaninsufficient Th1response,thusleadingtogranulomaformation.5_Other

theoriespostulatethatunderlyingvascularinjury,induced by lipid deposition in endothelial cells, might result in a granulomatousresponse.4

Autoimmunityisacommonfindinginpatientswith granu-lomatousPPD,beingreportedinmorethanhalfofthecases. The most frequent arehypothyroidism, ulcerative colitis, Sjögren’ssyndrome, and multiple sclerosis.3,8 _{Some cases}

presentpositiveself-immunitymarkerssuchasantinuclear antibodies,rheumatoid factor, andcryoglobulins, suggest-ingthatautoimmunitymightalsoplayaroleinthisvariant. Furtherstudiesarenecessary toestablish anassociation.3

Otherfrequentfindingsdescribedarearterialhypertension anddiabetesmellitus.2

Granulomatous PPD is an often asymptomatic, benign condition.2 _{Thus, treatment is usually reserved for the}

management of associated symptoms, such as pruritus, and for cosmetic reasons, in patients distressed by the appearance of their skin. Management of suspected trig-gerssuchasdyslipidemiaandthetreatmentofassociated

venousstasis,whenpresent,mightbebeneficial.2,3

Antihis-taminesareusedtocontrolpruritus;however,theydonot have aneffectonthecourse ofthedisease.A systematic review9_{ofthetherapeuticstrategiesforPPDdemonstrated}

that theuse oflocalsteroids, localcalcineurin-inhibitors, rutoside, high doses of ascorbic acid, colchicine, pentox-ifylline, phototherapy, and laser therapy yielded a good response. Cyclosporine A and others immunosuppressants canbeusedinrefractorydisease.Largersystematicstudies arenecessarytoassesstheeffectivenessofthetherapeutic strategies.

Financial

support

Nonedeclared.

Author’s

contribution

DanielaCarvajal,ClaudiaQuiroz,ClaudiaMoralesandJavier Fernández:Approvalofthefinalversionofthemanuscript; conceptionandplanningofthestudy;elaborationand writ-ingofthemanuscript;obtaining,analyzingandinterpreting the data; effective participation in research orientation; intellectualparticipationinpropaedeuticand/or therapeu-tic conduct of the cases studied; critical review of the literature;criticalreviewofthemanuscript.

Conflicts

of

interest

Nonedeclared.

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