PenochSchonleln urpura

key olnLs
1 PS ls a vascullLlc dlsease ofLen followlng a u8l usually affecLlng chlldren buL also seen ln adulLs
2 1he maln sysLems lnvolved are Lhe skln Cl LracL kldneys and [olnLs
3 ulagnosls ls conflrmed by skln or renal blopsy demonsLraLlng lgA deposlLlon
4 MosL recover fully wlLhouL lnLervenLlon buL recurrences are common
Lpldemlology MosL common ln chlldren wlLh over half less Lhan 3 yrs old ulsease ls more severe ln
older
chlldren and adulLs especlally wlLh regards Lo renal lnvolvemenL CfLen follows an upper resplraLory
lnfecLlon buL a recenL sLudy suggesLs LhaL an occulL mallgnancy may be Lhe cause
aLhophyslology CorrelaLlon wlLh recenL lllness suggesLs an anLlgenlc preclplLanL Lo Lhe ensulng
vascullLls
very slmllar Lo lgA nephropaLhy and wlll see lgA deposlLs ln skln and kldneys on blopsy lnLeresLlngly
pLs
wlLh renal lnvolvemenL also have lgC anLlbodles agalnsL mesanglal cells
SympLoms 4 classlc ones
1 8ash (96) purpurlc ofLen palpable dlsLrlbuLlon mosLly over lower exLremlLles May be urLlcarlal
and cause edema parLlcularly ln klds
2 ArLhralglas (61) knees and ankles are LargeLs noL a desLrucLlve arLhrlLls
3 Cl problems (48) cramplng abdomlnal paln ofLen wlLh vomlLlng Appears abouL a week or more
afLer Lhe rash beglns alLhough Lhere are cases where Cl problems occur wlLhouL a rash 23 have Cl8
and 30 have occulL blood loss Cn endoscopy you see pupurlc leslons +/ edema ulceraLlon or bowel
spasm
4 kldney dlsease (37) 8eglns few days Lo weeks afLer oLher sympLoms uA can show proLelnurla
(mlld) 88Cs and cellular casLs Many paLlenLs wlll be asympLomaLlc buL oLhers can develop nephroLlc
syndrome Cn blopsy can see mesanglal prollferaLlon and crescenL formaLlon severlLy of renal dlsease
ls correlaLed wlLh percenL of crescenL formaLlon
CLher organs less commonly lnvolved lungs and CnS
ulagnosls Susplclon from sympLomaLology should lead Lo blopsy of skln or kldneys for conflrmaLlon of
Lhe dlagnosls Skln blopsy shows leukocyLoclasLlc vascullLls wlLh lgA deposlLlon as seen by
lmmunofluorescence kldney blopsy slmllarly shows lgA deposlLlon
uux 1he blg ones are hypersenslLlvlLy vascullLls and cryoglobullnemla Should also conslder Wegener's
anLlC8M SLL and oLher vascullLlc syndromes
1reaLmenL MosL recover on Lhelr own wlLhouL LreaLmenL uapsone may help arLlcular Cl and skln
dlsease
ln Lhose wlLh renal dlsease parLlcularly lf lL ls severe sLerolds (meLhylprednlsolone followed by 3
monLhs
of oral prednlsone aL 1mg/kg/d) are ofLen glven CLher Lheraples (usually ln con[ucLlon wlLh sLerolds)
LhaL
have been Lrled are cyclophosphamlde azaLhloprlne plamapheresls and lvlC none have been sLudled
ln
randomlzed conLrolled Lrlals kldney LransplanL ls a flnal opLlon buL recurrenL dlsease can occur ln Lhe
new
kldney so one should walL 12 years unLll afLer Lhe rash dlsappears Lo LransplanL
rognosls ln chlldren preLLy good (over 93)! lnlLlal recovery occurred ln 89 of adulLs unforLunaLely
longLerm prognosls ln adulLs wlLh PS and kldney lnvolvemenL showed LhaL 11 were dlalysls
dependenL
and 13 had severe renal dlsease Why ls Lhls? robably due Lo some damage LhaL occurs ln Lhe kldneys
aL
Llme of dlsease whereby Lhe oLher glomerull parLlclpaLe ln hyperfllLraLlon" Lo malnLaln adequaLe renal
funcLlon Cver Llme Lhls can lead Lo flbrosls and glomerular damageACL lnhlblLors mlghL help!
Cne reLrospecLlve sLudy showed 13 year survlval of only 74 ln pLs wlLh PS wlLh carclnoma of Lhe Cl
or
resplraLory LracLs as Lhe leadlng cause of deaLh