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A cae of nodutrar cutaneous amyloldcis and rwiew of $e literature

Jonathan C_KonoFns*i nD- Sarah J Solrfar tD, Kerd L Ro6irl3 f,D, qatula iGu [D
Dem.tology &ilne JqrrrEl ,l (a): lo

Baylor Cdlege of Medicine, Houston, Ter6s

AEiract
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Nrvt| iln. fr ic t+B ElBt inm nt dirmB d rla|m F amlni.ln*iq The amrtnfot frlvile atr Na.A m nr* r rninr F l^ trll'.A
'NCA\ d
but arealso lhe pre\iaiting amylou component in gimary sysiernicarnylSidoais (PSA) arld myelotn&associated sy*fllicamyloi{rosis- Age
presentation in NCA has rarEed firyIl 20 to 87 yrs withort a &r
gender predilecilion. Ptwress]cn ftom NGA to primary sysb$ric arnyloircris
has been lEported, wilh an estimated liMime risk of appodmately 7 perent, promptiry the need fur appopriab follor up to e\,aluate fur the
pressrce of sysbrntc artyloforosis. l ,b report a case of nodular cutaneous amy'oidocb in an ofis'wbe heaffry 62-!reer-old \,rcman and vve
reviewthe librafure.

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(,E gu J - a# ;*H^- J dF. J# am.lail ar aa & id# lh^..^ J k *i- tur,|.iM k ldL.
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producsd arnyloid, brErd gmary cutaned,s amyhidoots {PCA). Hodubr artaneqrs amy,loi(h;is is the raGst lam of rcA. I E report herein a
case of nodularcutaneorF amybirosis in an drenrise healthy vrcEran and we rcview lhe literatre.

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Fig$e t. t.5 x 1, cm Vdkbwr tsqE e pa&r#s clEst *s.EI" prmch
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A 62-yirardd aufian rilitfi no significant pasl mdiral history prsfitgl with a nsi, le6bn on her cfied that she frst notlced 2 months fflor. She
denied any recent draege in size or col,r. E)m Evealed a 1.5 cm x 1.2 crn yelb\rFbro,rflri plaque on her medial chest (Figure 1). A 4 mm punch
biopy was perionned. Mir:roeooflc examination sirorecl amorphous, pinkishgray mabrial betnreen ihe collagen fiixrs in fie papilhry an<t
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findings, a dl@nosis of nodularcrtarEdl3aflryloi.rq{swas Gtatb. Serum and urireprdeinelecfrophoresie sfrrdiesrererEgatlve. I?Epatient
urasinfoflredoflheresultsandmadea\rethdregularfuftox.upwi0rherfirmrycareproviderrrasnecessarygiwnfiepoGsrit lityfur
preglB6sion d NCA te qBetnie Cisease. She hler tdumed breernphb xCsien ef tte pla+re. Beth tn dE to,Dridizalien prebeebr
immunog,obulin mRl{Aand lifuidcfiromatoEraphytandem mass spectometry pefbmd on peptjdG extracted ftom Congo red-positiveareas
of the specirn deE&d a peplide profile oonsidefit with AL 0amMa light cfiains) type amylcid deposit on.

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r IIE arrrJlsww- d9 s Yrwl, ur urwffio urd&Err4d u, urs %uawru@ \rcPUDrrMr vr flrwruuc alrgiEgalco vr riiloruucu Prurcrrro! wflcu(vct,
tenrEd amyl*r, ,nto various tissues dtle body" The amyh*d &poits trraqge in afl abnormd fbilhrbm ard fi}ry disruStissrre ardlihc&Ife
and fuft*ion. Amyldd appeaB as an amoryhous eosinophilic by ligtrt mioccopy ard s*dns pcihc for Co4o red. Addtisra[y,
'Ierbfi
sBecimens staind with eengp Id Ctnensheb appe grB6n bfueingsnce t*ren vblEC urxbr pelarbsC lilhtr I Eetu,B lhat hao beeeme ure
pld stardard for diqnosis.
Pri nary .utffBE amylok qris is a set of amyloidGs in $ftk,l amyloid is deposiH mly into tlte stifl ard indudes 3 distind entities: lichen
anylgiabsk, maqihJ amylcidoBis, alld naduhr Grtaneaus-amy,loiddBis (NCA). The rare6( dthese is NCA, in yhich amylfi difrrsdy irfilhates
lhe dermis, subqrtb, and blood vessel walls [tl. ln lidren and mawlar amyloidosis, ftc amyloid depooih & not invadc blood vessela or e*nd
blMkkb

The an4doirl fikils in ]tCA ate composerl d imr$mryt hdin ngfrt rEirls" Cnned "prE{Ein Al-- kdsin AL is not uni$ie !o NCA and is lhe
pre\.ailing arnylou cofiiponert in scveral drtr dnyloidocirt indr.rding primary sy*mic amyoi&dn (PSA) and rrydofira-associabd syslemic
amyloidd$s. lmmunohisfiodE nical stridie tla\re sho/n trat the lirht dlains in NCA may be of the kappa and/or lamb6 tlDe l2l. Local
rrcrodmal plasrm cdls pro<fue and secreb the light ctirs lhmrgh an unkrpum me<fianisrn, G demonstrabd by &ht chain restic{ion and
sdeGJbrgerE{csB,+l-Thisasincontretbtt:eamytk ftrilsinlicfsrarsmejlaraflrybi(hBis,whicharEsecrEtedtykeratirlosyt$and
comptlseO cmenyof cilioleratin 5
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