Fpch ^fthaj Jf` Old—Uaj 636* Gfm|fpy 0>>2
Ml~hpablmic Systloic Ei`pasis
—Sajaoam lt fj
64<
Cfsl Pl~apt
Ml~hpablmic Systloic Ei`pasis Oioickimb Imfffooftapy@plfst Cfpcimaof
Bfppam G- Sajaoam* OD; Ljiwf`lth R|* OD; ^f|j ^ltlp Paslm* OD
◁
Ml~hpablmic systloic st`pasis* ~pluia|sjy kmarm fsml~hpablmic st`pasimb dlpoa~fthy* is f mlrjy plcabmiwldsystloic st`pasimb disapdlp ~piofpijy feelctimb ~ftilmtsrith chpamic plmfj efij|pl- ^ftilmts rith skim imuajulolmtaetlm dlulja~ ~f~|jls fmd ~jfv|ls rith
~lf| d’apfmbl
s|p/efcl chfmbls- Thl jarlp lxtploitils fmd tp|mk fpl oastcaooamjy feelctld- Thl oast io~aptfmt histajabic dieelp/lmtifj difbmasis is rith scjlpaoyxldlof- Ta a|p kmarj/ldbl* rl pl~apt thl stpst cfsl ae ml~hpablmic systloic st/`pasis imuajuimb thl `plfsts ae f 56/ylfp/ajd raofm rithlmd/stfbl plmfj dislfsl* cjimicfjjy oioickimb imfffooftapy`plfst cfpcimaof- Rl ~pa~asl thft ml~hpablmic systloicst`pasis `l camsidlpld im thl dieelplmtifj difbmasis fs f pfpl~assi`ijity rhlm c|tfmla|s chfmbls im thl `plfst s|bblstimfffooftapy `plfst cfpcimaof im f ~ftilmt rith plmfj efij/|pl-(
Fpch ^fthaj Jf` Old-
0>>2;636764<–648)
M
l~hpablmic systloic st`pasis (MSE) is f plclmtjy dl/scpi`ld systloic st`pasimb disapdlp ~piofpijy feelct/imb ~ftilmts rith chpamic plmfj ims|estcilmcy- Fjtha|bh thllxfct cf|sl is |mkmarm* `aml ofppar–dlpiuld cipc|jftimbst`pacytls fpl s|s~lctld ta `l imuajuld im thl ~fthablm/lsis ae this pfpl camditiam- Skim imuajulolmt im MSE epl/v|lmtjy ~plslmts am thl lxtploitils fs lpythlofta|s ~f~/|jls ap ~jfv|ls* aetlm rith
~lf| d’apfmbl
s|pefcl chfmblsfmd raady imd|pftiam- Tp|mcfj imuajulolmt is jlss cao/oam* fmd `plfst imuajulolmt hfs mat ~pluia|sjy `llm pl/~aptld-Rl pl~apt hlpl f cfsl ae f raofm rith fdufmcld plmfjdislfsl rha ~plslmtld rith `ijftlpfj `plfst srljjimb jlfd/imb ta thl cjimicfj io~plssiam ae imfffooftapy `plfst cfp/cimaof- @ia~sy ae aml ae thl `plfsts plulfjld MSE-
PL^APT AE F CFSL
Thl ~ftilmt is f 56/ylfp/ajd raofm rith lmd/stfbl plmfj dis/lfsl am hloadifjysis rha ~plslmtld rith tlmsl srljjimb fmd‘‘dio~jimb’’ ae `ath `plfsts- Thl ~ftilmt’s oldicfj histapy fjsaimcj|dld dif`ltls oljjit|s* hy~lptlmsiam* fsthof* fmd hy~lpca/
Fccl~tld eap ~|`jicftiam Sl~tlo`lp 2* 0>>5-Epao thl Dl~fptolmt ae ^fthajaby fmd Jf`apftapy Oldiciml* MlrYapk/^pls`ytlpifm Has~itfj* Rlijj Capmljj Oldicfj Cajjlbl* Mlr Yapk(Dps Sajaoam fmd Paslm); fmd thl Dl~fptolmt ae ^fthajaby* MlrYapkOlthadist Has~itfj* @paakjym* MY (Dp R|)-Thl f|thaps hful ma pljlufmt stmfmcifj imtlplst im thl ~pad|cts apcao~fmils dlscpi`ld im this fpticjl-Pl~pimts7 Bfppam G- Sajaoam* OD* Plsidlmt im ^fthajaby* MlrYapk/^pls`ytlpifm Has~itfj*Rlijj Capmljj Oldicfj Cajjlbl* <0< L 58 St* Stfpp6>* Mlr Yapk* MY 6>>06 (l/ofij7 bfs9>60Nmy~-apb)-
fb|jf`ijity* rith f plclmt thpao`lctaoy ~lpeapold eap f jlet fpothpao`asis imuajuimb hlp fptlpiaulma|s sh|mt- Thl ~ftilmt rfstfkimb ofmy oldicftiams imcj|dimb l~altim fjef- Thl ~ftilmt’samjy sibmistcfmt jf`apftapy stmdimbs rlpl fm ljluftld cplftimiml(6>-0 ob!dJ)* f oijd mapoacytic mapoachpaoic fmloif (hloa/bja`im* 66-> b!dJ)* fmd oijd thpao`acyta~lmif (~jftljlt ca|mt*64>
6>
3
!
J)- Shl hfd ma ~fj~f`jl `plfst ofss am ~hysicfjlxfoimftiam- Thl cjimicfj io~plssiam rfs imfffooftapy `plfstcfpcimaof* eap rhich f `ia~sy rfs ~lpeapold- Thl plelppimb ~f/thajabist matld ‘‘fm imstjtpftimb ~fttlpm ae st`pa`jfstic cljjs’’ fmds|`oittld thl sjidls eap cams|jtftiam ta p|jl a|t st`paoftasis-
OFTLPIFJS FMD OLTHADS
Cjimicfj imeapoftiam rfs a`tfimld epao ~ftilmt plcapds- Thldifbmasis rfs `fsld am lxfoimftiam ae histajabicslctiamsstfimldrith hloftaxyjim/lasim- Stpaofj o|cim rfs dloamstpftld riththl Fjcifm `j|l histachloicfj ~pacld|pl* fmd CD58/plfctiul fmdCD34/plfctiul cljjs rlpl idlmtistld `y ioo|mahistachloistpy|s/imb f stfmdfpd fuidim/`iatim olthad-
^FTHAJABIC EIMDIMBS
Thl lxcisiamfj `ia~sy s~lciolm ae thl jlet `plfst olf/s|pld <->
4->
6-> co rith fm fttfchld skim ljji~slolfs|pimb <->
6-> co- Thl oicpasca~ic slctiams sharldofpkld thicklmimb ae thl dlpois rith fcc|o|jftiam aethick cajjfblm `|mdjls sl~fpftld `y cjlets im thl ~f~ijjfpyfmd pltic|jfp dlpois (Eib|pls 6 fmd 0)- Thlpl rfs eacfjlxtlmsiam imta thl s|`c|tfmla|s tiss|l fjamb thicklmld st/ `pa|s sl~tfl- Rithim thl dlpois thlpl rfs f jfoimfp ~pa/jielpftiam ae sofjj `jaad ulssljs s|ppa|mdld `y f ~pldao/imfmtjy ~jfsofcytic imstjtpftl (Eib|pl 3)- Hibhlp ofbmist/cftiam ae thl cajjfblma|s stpaof plulfjld fm imcplfsldm|o`lp ae s~imdjld cljjs plslo`jimb st`pa`jfsts fmd im/cplfsld stpaofj o|cim (Eib|pls 4 fmd <)- Thl stpaofjs~imdjl cljjs rlpl jfpbljy CD58/~asitiul fmd CD34/~asi/tiul (Eib|pl 5* f fmd `)-
CAOOLMT
Ml~hpablmic systloic st`pasis is fm lolpbimb dislfsl~pimci~fjjy feelctimb imdiuid|fjs rith fdufmcld chpamicplmfj dislfsl-
6
It hfs fjsa `llm fssaciftld rith chpamic hl/~ftic dislfsl fmd thl hl~ftaplmfj symdpaol-
0
Thl scjlpa/dlpof/jikl c|tfmla|s ofmielstftiams ae this cjimica~ftha/jabic lmtity rlpl stpst plcabmiwld im 6992 im f cahapt aeplmfj difjysis ~ftilmts fmd pl~aptld `y Car~lp fmd caj/jlfb|ls
3
im 0>>>- Thl tlpo
ml~hpablmic st`pasimb dlpoa~fthy
(MED) rfs imtpad|cld im 0>>6-
4
S|`slv|lmtjy* fm f|ta~sy~lpeapold am fm imdiuid|fj difbmasld rith MED pl/ulfjld st`pasis ae thl dif~hpfbo* ~safs o|scjl* plmfj t|/ `|jls* fmd pltl tlstis* s|bblstimb thft thl st`pasimb disap/dlp rfs mat jioitld lxcj|siuljy ta thl imtlb|olmt-
<
Fd/
645
Fpch ^fthaj Jf` Old—Uaj 636* Gfm|fpy 0>>2
Ml~hpablmic Systloic Ei`pasis
—Sajaoam lt fj
Eib|pl 6-
Jar/~arlp iofbl sharimb st`pacajjfblma|s thicklmimb ae thl dlpois (hloftaxyjim/lasim* apibimfj ofbmistcftiam
0>)-
Eib|pl 0-
Oldi|o/~arlp iofbl sharimb thl fcc|o|jftiam ae thick cajjfblm `|mdjls sl~fpftld `y cjlets im thl pltic|jfp dlpois (hloftax/yjim/lasim* apibimfj ofbmistcftiam
4>)-
Eib|pl 3-
Hibh/~arlp iofbl sharimb f ~lpiufsc|jfp* ~pldaoimfmtjy ~jfsofcytic imstjtpftl im thl dlpois (hloftaxyjim/lasim* apibimfj ofb/mistcftiam
4>>)-
ditiamfj pl~apts dloamstpftld thft thl j|mbs* hlfpt* fmdskljltfj o|scjl cfm fjsa `l feelctld*
5*2
fmd fs f camsl/v|lmcl* thl ~plelppld tlpoimajaby is mar MSE!MED-C|pplmtjy* 62< cfsls hful `llm pl~aptld ta thl Imtlp/mftiamfj Clmtlp eap Ml~hpablmic Ei`pasimb Dlpoa~fthyPlslfpch-
8
Ml~hpablmic systloic st`pasis!ml~hpablmic st/ `pasimb dlpoa~fthy is mat plstpictld ta ~ftilmts |mdlp/baimb difjysis eap chpamic plmfj ims|estcilmcy* fmd thl tlp/oimajaby ~pa~asld `y Giolmlw fmd carapklps*
5
‘‘difjysis/fssaciftld systloic scjlpasis*’’ is ma jamblp plcaoolmdld-Cjimicfj io~paulolmt im MSE!MED ofy acc|p im cfslsrhlpl thl plmfj dyse|mctiam is s|cclsse|jjy plulpsld—|s|fjjy lfpjy im thl ca|psl ae thl dislfsl `leapl camtpfc/t|pl dlulja~olmt- Thlpleapl* it is io~aptfmt eap ~hysicifmsta `l frfpl ae this dislfsl simcl lfpjy difbmasis ofy jlfdta `lmlstcifj imtlpulmtiam-Ml~hpablmic systloic st`pasis!ml~hpablmic st`pasimbdlpoa~fthy feelcts ofjl fmd elofjl ~ftilmts lv|fjjy* hfsma f~~fplmt pfcifj ~pldijlctiam* fmd hfs `llm dac|olmtldim ~ftilmts epao 8 ta 85 ylfps ae fbl-
6
Oast ae thl cfslpl~apts sa efp hful `llm epao thl \mitld Stftls* `|t athlpcfsls hful `llm dac|olmtld im L|pa~l* Fsif* Imdif* fmdthl Oiddjl Lfst-
9
Plmfj ims|estcilmcy is |miulpsfj im MSE!MED ~ftilmts fmd ofy `l fc|tl ap chpamic- Io~aptfmtjy*6>, ae ~ftilmts rith MSE!MED hful mlulp `llm dif/jywld-
6>
Thl cf|sls ae thl |mdlpjyimb plmfj dislfsl imMSE!MED ~ftilmts fpl ufpif`jl- Thl amslt ae MSE!MEDofy `l tild ta athlp cjimicfj lulmts- Eap lxfo~jl* ofmy~ftilmts dlulja~imb MSE!MED pl~apt f plclmt thpao`aticlulmt im thl ~plcldimb 0 rllks-
6
F sofjj s|`slt ae thlsl~ftilmts hful fmticfpdiaji~im fmti`adils-
66
Fjsa* fm imcitimbpajl eap s|pblpy hfs `llm ~ast|jftld `lcf|sl ofmy ~f/tilmts rith MSE!MED (|~ ta 48,) hful hfd s|pbicfj ~pa/cld|pls* aetlm im thl 0 rllks ~piap ta thl amslt ae MSE!MED-
6
Fm ljluftld lpythpacytl sldiolmtftiam pftl ap C/plfctiul ~patlim rfs ea|md im 9 ae 60 ~ftilmts im amlst|dy-
60
Fjtha|bh thl ~fthablmlsis ae MSE!MED is imcao~jltljy|mdlpstaad* it is tha|bht thft `aml ofppar–dlpiuld cip/c|jftimb st`pacytls fpl f`lppfmtjy plcp|itld ta skim fmdathlp tfpblt tiss|ls* rhlpl thly lmbfbl im dysplb|jftldoftpix ~pad|ctiam fmd ploadljimb-
6
Slulpfj f|thaps hfulcamcj|dld thft TBE/@6 is thl cytakiml pls~amsi`jl eap thlst`patic ~paclss-
6*60
It hfs `llm matld thft ma cfsls ae MSE!MED rlpl idlmtistld ~piap ta 6992- Car~lp fmd caj/jlfb|ls
63
s|s~lct dl~asitiam ae f plclmtjy imtpad|cld of/tlpifj* s|ch fs f camtpfst fblmt* oldicftiam* ap athlp fjjlp/blm im ~lpi~hlpfj tiss|ls fs thl imcitimb lulmt-^ftilmts rith MSE!MED ~plslmt rith ~fime|j ap ~p|pit/ic* syooltpic* skim/cajapld ta lpythlofta|s ~f~|jls caf/jlscimb imta `pfrmy ~jfv|ls—|s|fjjy am thl lxtploitilsfmd tp|mk- Fmkjls* shims* fmd jarlp thibhs fpl thl oastcaooamjy feelctld sitls-
60
Thl skim jlsiams aetlm tfkl amf
~lf| d’apfmbl
f~~lfpfmcl* fmd ~ftilmts fpl aetlm oistfk/lmjy difbmasld fs hfuimb cljj|jitis- Thl cjimicfj dieelplm/tifj difbmasis fjsa imcj|dls scjlpadlpof* lasima~hijic efs/ciitis* lasima~hijif/oyfjbif symdpaol* fmd scjlpaoyxldl/of- Ofmy ~ftilmts rith MSE!MED fpl ea|md ta hful ac/|jfp jlsiams dlscpi`ld fs yljjar scjlpfj ~jfv|ls-
64
F sofjj~lpclmtfbl (
<,) ae MSE ~ftilmts hful f e|joimfmtca|pslchfpfctlpiwld `y f pf~id jass ae oa`ijity fmd slulpl ~fim-
6
Thl acc|pplmcl ae
~lf| d’apfmbl
s|pefcl chfmbls am thl `plfsts ae thl elofjl ~ftilmt im this pl~apt jld ta thl cjim/icfj io~plssiam ae imfffooftapy `plfst cfpcimaof- Ta a|pkmarjldbl* this is thl stpst cfsl ae MSE!MED ~plslmtimbim this ofmmlp-Skim `ia~sy is camsidlpld thl bajd stfmdfpd im thl di/fbmasis ae MSE!MED- Hista~fthajabic stmdimbs imcj|dl fofpkldjy thicklmld pltic|jfp dlpois rith f disapdlpjy fp/pfmblolmt ae cajjfblm st`lps fmd s|ppa|mdimb cjlets- Thl
Fpch ^fthaj Jf` Old—Uaj 636* Gfm|fpy 0>>2
Ml~hpablmic Systloic Ei`pasis
—Sajaoam lt fj
642Eib|pl 4-
Hibh/~arlp iofbl ae thl dlpois sharimb s~imdjld cljjs plslo`jimb st`pa`jfsts (hloftaxyjim/lasim* apibimfj ofbmistcftiam
4>>)-
Eib|pl <-
Imcplfsld stpaofj o|cim is sllm im thl dlpois (Fjcifm `j|l* apibimfj ofbmistcftiam
4>>)-
Eib|pl 5-
Ioo|mastfims eap CD58 (f) fmd CD34 (`)- Ofmy ae thl s~imdjld cljjs rlpl ~asitiul eap CD58 fmd CD34 (ioo|ma~lpaxidfsl* apibimfj ofbmistcftiam
0>>)-
~paclss aetlm lxtlmds imta thl s|`c|tfmla|s tiss|l fmd!ap o|scjl fjamb st`pa|s sl~tfl- Thlpl ofy `l imcplfsldstpaofj o|cim fmd mlaufsc|jfpiwftiam im thl dlpois- Foijd ~lpiufsc|jfp jyo~ha~jfsofcytic imstjtpftl is mat |m/caooam- Ioo|mahistachloistpy plulfjs imcplfsld m|o/ `lps ae CD34/~asitiul* ~pacajjfblm/~pad|cimb st`pa`jfstsfmd CD58/~asitiul cljjs- Thl skim `ia~sy stmdimbs oastcjasljy plslo`jl scjlpaoyxldlof; harlulp* this camditiamcfm `l p|jld a|t am cjimicfj bpa|mds simcl thl jfttlp is matty~icfjjy fssaciftld rith plmfj dislfsl* is oast caooamam thl efcl fmd mlck* fmd acc|ps im ~ftilmts rith fm |m/dlpjyimb ~fpf~patlimloif (|s|fjjy ioo|mabja`|jim B)-
6<
Thl tplftolmt eap MSE!MED is ufpif`jl fmd imcj|dlsplmfj tpfms~jfmtftiam* lxtpfcap~aplfj ~hata~hlplsis* ~jfs/of~hlplsis* fmd capticastlpaids* foamb athlps- Jl@ait
65
s|bblstld pld|cimb thl dasl ae lpythpa~ailtim im ~ftilmtsrith plmfj dyse|mctiam simcl plcao`imfmt lpythpa~ailtimhfs ~atlmtifj st`pablmic ~pa~lptils- Thl a~tiofj ofmfbl/olmt is mat kmarm simcl tplftolmt dftf fpl fufijf`jl eapamjy f sofjj m|o`lp ae cfsls- E|pthlpoapl* it is diestc|jtta fsslss cjimicfj io~paulolmt* fs ma stfmdfpd eap stfbimbthl dislfsl lxists- ‘‘Io~paulolmt’’ blmlpfjjy io~jils pl/d|ctiam ae imd|pftiam* pld|ctiam ae camtpfct|pls* ap io/~pauld oa`ijity-
6
Eajjar/|~ skim `ia~sy fetlp tplftolmt issljdao |mdlptfklm-Thl tlpo
imfffooftapy `plfst cfpcimaof
rfs ~pa~asld im6904 ta dlscpi`l thl cjimicfj f~~lfpfmcl ae ~ftilmts riththis camditiam-
62
Imfffooftapy cfpcimaof is chfpfctlpiwld `y lpythlof ae thl ofoofpy skim rith thicklmimb* ls/~lcifjjy ft thl ldbl ae thl lpysi~ljaid fplf-
68
Thl skim ofyshar
~lf| d’apfmbl
chfmbls* rhich fpl aetlm oast ~pa/ma|mcld im thl dl~lmdlmt ~aptiams ae thl `plfst- Im fd/ufmcld cfsls thlsl c|tfmla|s ofmielstftiams ofy lxtlmdta thl chlst rfjj- Ty~icfjjy* thl `plfst is lithlp diee|sljyimd|pftld ap f ofss cfm `l ~fj~ftld-
69
Thl dieelplmtifjdifbmasis ae thl cjimicfj ~plslmtftiam ae imfffooftapy `plfst cfpcimaof imcj|dls ofmy mammla~jfstic camditiamss|ch fs cljj|jitis* dll~ ulma|s thpao`asis* pfdiftiam dlp/oftitis* ~asts|pbicfj dlpofj jyo~hldlof*
0>
ofstitis d|lta ufpia|s cf|sls* scjlpadlpof* fmd ~fmmic|jitis- Jyo~ha/ofta|s fmd jl|kloic imstjtpftiam ofy fjsa oioic imfffo/oftapy cfpcimaof- Im ~ftilmts rith plmfj efij|pl* MSE!MED sha|jd `l camsidlpld fs f pfpl ~assi`ijity rhlm c|/tfmla|s chfmbls im thl `plfst s|bblst imfffooftapy`plfstcfpcimaof- Fm f~~pa~piftl s|pbicfj `ia~sy ae thl skim fmd|mdlpjyimb `plfst is mlclssfpy ta distimb|ish MSE!MEDepao imfffooftapy cfpcimaof-
NSF Mimicking Breast Carcinoma
Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a newly recognized systemic fibrosing disorder primarily affecting patients with chronic renal failure. Patients with skin involvement often develop papules and plaques with peau d’orange surface…
(More)
267
Reads
1
Readcasts
0
Embed Views
Recommended
More From This User
Notes
Load more
