AnBrasDermatol.2020;95(3):340---342
Anais
Brasileiros
de
Dermatologia
www.anaisdedermatologia.org.brCASE
REPORT
A
case
of
facial
atrophic
sarcoidosis
in
an
adolescent,
successfully
treated
with
the
combination
of
prednisone
and
hydroxychloroquine
夽,夽夽
Xiaomei
Zhu
,
Jianfang
Sun
∗DepartmentofPathology,InstituteofDermatology,ChineseAcademyofMedicalSciences,PekingUnionMedicalCollege,Nanjing, China
Received23December2018;accepted4August2019
Availableonline19March2020
KEYWORDS Adolescent; Atrophy; Hydroxychloroquine; Prednisone; Sarcoidosis
Abstract Sarcoidosisisamultisystemgranulomatousdisorderofunknownaetiology.Cutaneous
involvementoccursinupto30%ofpatients andskinfindingsareoftentheinitialpresenting
symptom.Thefacialatrophicformofsarcoidosiswithoutassociatedulcerationinadolescents
hasrarelybeendescribedintheliterature.Wereportacaseof13-year-oldmalepatientwith
afacialatrophicsarcoidosiswhowassuccessfullytreatedwiththecombinationofprednisone
andhydroxychloroquine.
©2020SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.Thisisan
openaccessarticleundertheCCBYlicense(http://creativecommons.org/licenses/by/4.0/).
Introduction
Sarcoidosisisacommonsystemic,noncaseating granuloma-tousdisease of unknownaetiology.Cutaneous sarcoidosis, the‘‘great imitator’’,can baffle cliniciansbecause of its diverse manifestations and its ability to resemble both
夽 Howtocitethisarticle:ZhuX,SunJ.Acaseoffacialatrophic
sarcoidosisinanadolescent,successfullytreatedwiththe combi-nationofprednisoneandhydroxychloroquine.AnBrasDermatol. 2020;95:337---9.
夽夽StudyconductedattheDepartmentofPathology,Instituteof
Dermatology,ChineseAcademyofMedicalSciences,PekingUnion MedicalCollege,Nanjing,China.
∗Correspondingauthor.
E-mails:626469575@qq.com(X.Zhu),fangmin5758@aliyun.com
(J.Sun).
common and rare cutaneous diseases. Morphologically, plaquesandpapulesarethemostcommonlyobserved cuta-neous lesions.1 Rare presentations including psoriasiform,
ichthyosiform, erythroderma, andatrophic and ulcerative forms have been reported.2 Histopathologically, the
typi-calfeaturesofsarcoidosisarenakedgranulomaswithfew inflammatorycells.3
Case
report
A 13-year-old man presented with a 2-year history of multiple depressed skin lesions on his face. The lesions initiallypresented asasymptomaticerythematouspatches whichhadgraduallydevelopeddepressedcentresovertime. There wasnohistoryof trauma, ulceration, fever, cough, breathlessness, sensory loss, or intralesional steroid used totreat thelesions.Beforepresentingtoourhospital,he https://doi.org/10.1016/j.abd.2019.08.028
0365-0596/©2020SociedadeBrasileiradeDermatologia.PublishedbyElsevierEspa˜na,S.L.U.ThisisanopenaccessarticleundertheCC BYlicense(http://creativecommons.org/licenses/by/4.0/).
Acaseoffacialatrophicsarcoidosisinanadolescent 341
Figure1 (A)Clinical presentationatthefirst visit. Multiple,well-defined,erythematous plaquesinvolvingtheface.(B)The
lesionsaredepressedwithacliffdropborder.(C)Granulomatousreactionpatterncharacterized bymultiplegranulomasinthe
upperdermis(Haematoxylin&eosin,×50).(D)Epithelioidcellgranulomas,withoutcentralnecrosisinassociationwithasparse
lymphocyticinfitrate(Haematoxylin&eosin,×200).(E)Clinicalpresentationattheendoftreatment,showingthatmostofthe
342 ZhuX,SunJ had been diagnosed as lupus vulgaris in another hospital
andhadbeen givenantituberculoustherapyfor6months. However,theskin lesionshad graduallyprogressedduring treatment. On physical examination, multiple erythema-tousplaqueswithaslightlyatrophicappearancewereseen onthe patient’s face (Fig.1A and B). A full neurological examination was carried out, and the patient was found tohave intact sensation. There wasno palpable enlarge-ment of peripheral nerves. Laboratory findings, including blood and urine routine examination, biochemistry inves-tigationsandantinuclearantibodytest,werewithinnormal limits.Purifiedproteinderivativetestprovedtobenegative, andaposterior-anteriorchestX-rayrevealedbilateralhilar lymphadenopathy.The skin biopsyshowed dermal numer-ouscompactepithelioidgranulomas,surroundedbyacollar ofsparselymphocyteswithnoevidenceofacid-fastbacilli orfungi (Fig.1Cand D).Therewasnopolarizable foreign material withinthe granulomas. We finallydiagnosed the patientwithatrophiccutaneoussarcoidosis.Thepatientwas started on 0.5mg/kg/day of prednisone (25mg/day) and 6.0mg/kg/day of hydroxychloroquine (300mg/day). After 2months,partiallyregressionoftheerythematousplaques andthebilateralhilarlymphadenopathywasachieved. Pred-nisonewastaperedby5mg/monthandwasdiscontinued4 monthslater. Then, oral administrationof hydroxychloro-quine was given for the consecutive 3 months. After 9 months of treatment, the patient presented an almost complete clinical response without bilateral hilar lym-phadenopathy(Fig.1EandF).
Discussion
Cutaneous sarcoidosis with atrophic-appearing lesions is uncommonand hasprimarily beenreportedin association withulcerativelesions,mostlyonthelowerlegs ofyoung andmiddle-agedpeople.2,4Thefacialatrophicformof
sar-coidosis without associated ulceration in adolescents has rarely been describedin theliterature. Inthe absenceof adefinitivediagnostictest for sarcoidosis,mostcases are diagnosedbyhistologyandexclusionofothercausesof gran-ulomatousinflammation.Majordifferentialdiagnosesofthe presentcasewere:leprosy,systemiclupuserythematosus, and lupus vulgaris. Clinically, leprosy may spontaneously resolve,leavingatrophicskin.Diagnosisofleprosyismade bydemonstrationofacid-fastbacilliinskinbiopsyorsmear, clinical symptoms and neural involvement. The diagno-sis of systemic lupus erythematosus could be ruled out because antinuclear antibodies were negative and there were no systemic symptoms. Ineffective antituberculous therapy could exclude lupus vulgaris. Histopathologically, sarcoidalgranulomascanalsobeseeninmultipleentities, includingforeignbodyreactions, infections, and immuno-deficiencysyndromes.5Itisimportanttoruleoutinfectious
organismsandforeignsubstances.Inourpatient,the diag-nosisofsarcoidosiswassupportedbythepresenceofhilar lymphadenopathy and histopathological findings. The rar-ity of cutaneous atrophic sarcoidosis makes it difficult to perform rigorous clinical trials and most of the informa-tion regarding treatments comes from case reports. The ulcerative-atrophic lesions tend to heal by using combi-nation therapy with prednisone, hydroxychloroquine, and eithermycophenolatemofetilorthalidomide.4 Althoughit
hadbeenreportedthattheatrophicformwasresistantto treatment,6ourcaserespondedwell.
In conclusion, we diagnosed and curatively treated a patient with cutaneous atrophic sarcoidosis. In such patients, the long-course combination therapy with pred-nisone and hydroxychloroquine may achieve the clinical cure.
Funding
source
CAMSInnovationFundforMedicalSciences (CIFMS-2017-I2M-1-017).
Authors’
contributions
XiaomeiZhu:Approvalofthefinalversionofthemanuscript; elaborationandwritingofthemanuscript;obtaining, anal-ysis,andinterpretationofthedata;effectiveparticipation in research orientation; critical review of the literature; criticalreviewofthemanuscript.
Jianfang Sun: Approval of the final version of the manuscript;criticalreviewoftheliterature;criticalreview ofthemanuscript.
Conflicts
of
interest
Nonedeclared.
References
1.NoeMH,Rosenbach M.Cutaneoussarcoidosis.CurrOpinPulm Med.2017;23:482---6.
2.HaimovicA,SanchezM,JudsonMA,PrystowskyS.Sarcoidosis:a comprehensivereviewandupdateforthedermatologist:PartI. Cutaneousdisease.JAmAcadDermatol.2012;66:699.e1-18. 3.CardosoJC,CravoM,ReisJP,TellecheaO.Cutaneous
sarcoido-sis: a histopathological study.J EurAcad Dermatol Venereol. 2009;23:678---82.
4.YooSS,MimouniD,NikolskaiaOV,KoubaDJ,SauderDN,Nousari CH.Clinicopathologicfeaturesofulcerative-atrophicsarcoidosis. IntJDermatol.2004;43:108---12.
5.WanatKA,RosenbachM.Apracticalapproachtocutaneous sar-coidosis.AmJClinDermatol.2014;15:283---97.
6.VinayK,DeD,HandaS,SaikiaUN.Generalizedatrophicplaques inayoungmale.Generalizedatrophicsarcoidosis.IntJDermatol. 2016;55:e56---8.
