Appr

oacht
ocaseofer
ythr
oder
ma

 Pr
esent
er–Dr
.Maheshwar
i

 Moder
ator–Dr
.Shi
l
pa.
K

 3rdmay2018

Sy
nony
ms

 Exf
oli
ati
veder
mat
it
is

 Exf
oli
ati
veer
ythr
oder
ma

 Redmansy
ndr
ome

Gener
ali
sedinf
lammat or
ydi
sor
derofski
nmani
fest
ingwi
ther
ythemaandscal
i
ngaf
fect
ing
>90%ofskinsurface.

Hi
stor
y

 Ter
m er
ythr
oder
ma–1868byHEBRA

 Basedoncl
i
nical
cour
se,
classi
fi
edi
nto

 Chr
oni
cal
l
yrel
apsi
ng(
Wison–Br
ocq)

 Chr
oni
cal
l
yper
sist
ing(
Hebr
a)

 Sel
fli
mit
ingepi
demi
cs(
Sav
il
l)

 Subdi
vi
sionsnol
ongerused.

Cl
assi
fi
cat
ion

1.Pr
imar
y-Ar
isi
ngonnor
mal
looki
ngski
n

o Duet
ounder
lyi
ngsy
stemi
cdi
sor
derordr
ugr
eact
ion

2.Secondar
y-Ar
isi
ngf
rom pr
eexi
sti
ngder
mat
osi
s.MC>50%

Epi
demi
ology

 I
nci
dence–0.
9–71per1,
00,
000pat
ient
s

 Mal
e:f
emal
e–2:
1to4:
1

 Anyagegr
oupcanbeaf
fect
ed

 Mc–41–61y
ear
s

 Rar
einchi
l
dren

 Occur
sinal
lraces
Cl
ini
calpr
esent
ati
on

Fourkeypoi
nts

1.Pat
chyeryt
hemabecomesuniv
ersal
over24-
48hr
s,accompani
edby
mal
aise,
shi
ver
ingandpy
rexi
a.

2.Scal
i
ngappear
s2-
6day
slat
er,
whent
heski
nishot
,dr
y,r
edandt
hickened.

3.Ski
nfeel
sti
ghtandi
tchy
,pat
ientf
eel
scol
d.

4.Scal
pandbodyhai
risev
ent
ual
l
lyl
ost
,nai
l
sbecomet
hickenedorshed.

Acut
eer
ythr
oder
ma-Cl
i
nical
–pl
atel
i
kel
argescal
es

Chr
oni
cer
ythr
oder
ma

 Smal
l
erscal
es

 Ski
nindur
ati
onduet
oedemaandl
i
cheni
fi
cat
ion

 Ect
ropi
onandepi
phor
a–secondar
ytochr
oni
cper
ior
bit
aledema.

 Pal
mo–pl
ant
arker
atoder
maseeni
n80%pt
s.

Di
seasesAssoci
atedwi
thExf
oli
ati
veDer
mat
it
is

 Der
mat
oses

 Sy
stemi
c

 I
nfect
ions

 Mal
i
gnancy

 Mi
scel
l
aneous/i
diopat
hic

CausesofAcut
eer
ythr
oder
ma

 Dr
ugr
eact
ions

 PRP

 Abr
uptwi
thdr
awl
ofst
eroi
dsi
npsor
iasi
s

 Toxi
cshocksy
ndr
ome

 REDD(
Recal
cit
ranter
ythemat
ousdesquamat
ingdi
sor
deri
nadv
ancedHI
V)

 St
aphy
lococal
superAgmadi
ated.

Commoncauses

 Eczema
  Psor
iasi
s

 Ly
mphoma/
leukemi
a/sezar
ysy
ndr
ome

 Dr
uger
upt
ions

 I
diopat
hic

DRUGSASSOCI
ATEDWI
THERYTHRODERMA

• Al
l
opur
inol

• Bet
a-l
act
am ant
ibi
oti
cs

• Car
bamazepi
ne/
oxcar
bazepi
ne

• Gol
d

• Phenobar
bit
al

• Pheny
toi
ns

• Sul
fasal
azi
ne

• Sul
fonami
des*

• Zal
cit
abi
ne

Pat
hogenesi
s

I
ntr
icat
eint
eract
ionofcy
toki
nes,
cel
l
ularadhesi
onmol
ecul
es(
IL-
1,2,
8,
ICAM-
1)andTNF-
α

• I
ncr
easedepi
der
mal
tur
nov
err
ate

• I
ncr
easedmi
tot
icr
ateandnumberofger
minat
iveski
ncel
l
s

• Decr
easedt
imer
equi
redf
orcel
l
stomat
ureandt
rav
elt
hroughepi
der
mis

• Lossofepi
der
mal
mat
eri
al+si
gni
fi
cantpr
oti
en,
nucl
eicaci
dsf
olat
eint
hef
orm of
scal
e

Di
ff
erentet
iol
ogyhav
eski
nsel
ect
iver
ecr
uit
mentofi
nfl
ammat
orycel
l
s.

Sezar
ysy
ndr
ome–Thel
per2cy
toki
nepr
ofi
l
e–CCR4ov
erexpr
essi
on

Psor
iasi
sandAD-ci
rcul
ati
ngadhesi
onmol
ecul
esI
CAM-
1,VCAM-
1,E-
sel
ect
in

i
mmunosuppr
essi
onandi
nfect
ion

Cl
ini
calf
eat
ures

• Pr
uri
tusseeni
n90%pat
ient
s
 • Sev
erei
nder
mat
it
isandsezar
ysy
ndr
ome.

• Chr
oni
cit
ch–l
i
cheni
fi
cat
ion(
1/3cases)

Classi
ccl i
nicalfeat
ures–ery
themat
ouspatchest
hati
ncr
easei
nsi
zeandcoal
escei
nto
general
iseder yt
hemawi t
hshi
nyappear
ance.

Acut
efor
m –l
argepl
atel
i
kescal
es

Chr
oni
c–smal
ldr
yscal
es

Dependi
nguponnat
ureofscal
e

• Fi
ne–ADorder
mat
ophy
tosi
s

• Br
anl
i
ke–sebor
rhei
cder
mat
it
is

• Cr
ust
ed–pemphi
gusf
oli
aceous

• Exf
oli
ati
ve–dr
ugr
eact
ions

Fev
erwi
thshi
ver
ing,
mal
aise

Ski
n–br
ightr
ed,
dry
,scal
y,hotandi
ndur
ated,
feel
i
ngoft
ight
ness.

Pav
ithr
an’
snosesi
gn:

• Compl
eteabsenceofscal
i
ngander
ythemaofnoseandper
inasal
areas.

• Hy
pot
hesi
s-gr
eat
ersunexposur
eofnose

Rai
sedmet
abol
i
crat
es

• poi
ki
lot
her
mia

• hy
poal
bunemi
a,edema

• negat
iveni
tr
ogenbal
ance muscl
ewast
ing

• pr
otei
nlossent
eropat
hy

Ey
e–B/
lect
ropi
on,
epi
phor
a,pur
ulentconj
unct
ivi
ti
s.

Hai
r–di
ff
useef
fl
uvi
um,
scal
i
ngofscal
p,ci
cat
ri
cial
alopeci
a

PPK–30%ofer
ythr
oder
mapat
ient
s,Of
tenseenasear
lysi
gni
nPRP

Cr
ust
edker
atoder
ma–Nor
wegi
anscabi
es

Pai
nful
andf
issur
edker
atoder
ma–Sezar
ysy
ndr
ome.

Nai
ls–40%pat
ient
s

Pr
eexi
sti
ngder
mat
oses,
nai
lchangespr
eceder
ythr
oder
ma
Pi
tsi
npsor
iasi
sorhor
izont
alr
idgi
ngi
nAD,
other
sdev
elopsubsequent
ly.

Dull
ness,di
scol
orat
ion,
bri
tt
leness,
ony
chol
ysi
s,subungual
hyper
ker
atosi
s,par
ony
chi
a,
beau’
sli
nes,spl
i
nterhemor
rhages.

Shor
eli
nes–al
ter
nat
ingbandsofnai
lpl
atedi
scont
inui
ty.

• Repr
esentdr
ugi
nduceder
ythr
oder
ma

• Li
nesr
efl
ect
stheper
iodsoft
imedr
ugwasused.

CLUESFORCOMMONUNDERLYI
NGDI
SEASE

Psor
iasi
s

• Hi
stor
yofl
ocal
i
zeddi
sease

• Per
sonal
orf
ami
l
yhi
stor
yofpsor
iasi
s

• Cl
assi
cpsor
iasi
for
m pl
aquesonel
bows,
knees,
sacr
um,
etc.

• Wel
l
-ci
rcumscr
ibedpl
aquesatmar
ginsofer
ythr
oder
ma

• Lar
gel
amel
l
arscal
es

• Psori
ati
cnail
changes:
pit
s,oi
ldr
opsi
gn,
ony
chol
ysi
s,subungual
hyper
kerat
osi
s

• Col
l
aret
tesofscal
esuggest
iveofr
upt
uredpust
ulesofpust
ularpsor
iasi
s

• Psor
iat
icar
thr
it
is

• Withdr
awalofcor
ti
cost
eroids,
methotr
exat
eorcy
clospor
ine(
alsoef
ali
zumab
pr
iortoit
sremovalf
rom t
hemar ket
)

HPE

• Psor
iasi
for
m epi
der
mal
hyper
plasi
a

• Conf
luentpar
aker
atosi
slay
eredwi
thneut
rophi
l
s

• Spongi
for
m pust
ules

• Hy
pogr
anul
osi
s

• Di
l
atedt
ort
uouspapi
l
lar
ybl
oodv
essel
s

At
opi
cder
mat
it
is

• Hi
stor
yofl
ocal
i
zeddi
sease

• Personal
orfamil
yhi
stor
yofat
opy(
atopi
cder
mat
it
is,
ast
hma,
rhi
noconj
uncti
vi
ti
s)

• Mar
kedpr
uri
tus
 • Cl
assi
cat
opi
clesi
onsi
nant
ecubi
tal
andpopl
i
teal
fossae

• Li
cheni
fi
cat
ionandpr
uri
gonodul
ari
s

• Excor
iat
ions

• Focal
ski
nat
rophyf
rom y
ear
sofuseofpot
entt
opi
cal
ster
oids

• El
evat
edser
um I
gE,
eosi
nophi
l
ia

HPE

• Mi
l
dtomoder
ateacant
hosi
s

• Var
iabl
espongi
osi
s

• Der
mal
eosi
nophi
l
s

• Par
aker
atosi
s

Dr
ugr
eact
ions

• Recentst
artofnewdr
ug,
oruseoff
requent
lyi
mpl
i
cat
eddr
ug

• Rapi
donsetofr
ashandpr
ogr
essi
on

• Pr
ecededbymor
bil
l
ifor
m orscar
lat
ini
for
m exant
hem

• Ly
mphadenopat
hy,
Organomegal
y,Fev
er

• Nohi
stor
yofski
ndi
seases

• Er
ythema1st–f
lexur
es

• Bestpr
ognosi
s

• Ant
iconv
ulsant
s:st
eri
l
epust
ulosi
s.

• Usuall
yresol
veswithi
n2–6weeksaf
terwi
thdr
awal
ofr
esponsi
ble
dr
ug;possibl
eexcepti
oni
sDI
HS/DRESS

HPE

• Per
ivascul
ari
nfi
l
trat
ewi
theosi
nophi
l
s,Li
chenoi
dinf
il
tr
ate

• Necr
oti
cker
ati
nocy
tesatal
ll
evel
sofepi
der
mis

• Vacuol
ardegener
ati
onofbasal
lay
er

I
diopat
hicer
ythr
oder
ma

• AkaRedmansy
ndr
ome

• El
der
lymen
 • Chr
oni
c,r
elapsi
ng,
Sev
erepr
uri
tus

• Pal
mopl
ant
arker
atoder
ma

• Der
mat
opat
hicl
ymphadenopat
hy

LESSCOMMONCAUSES

Cut
aneousT-
cel
lly
mphoma

• Sézar
ysy
ndr
omeMCt
haner
ythr
oder
micmy
cosi
sfungoi
des

• I
ntensepr
uri
tus

• Deeppur
ple–r
edhue

• Pai
nful
,fi
ssur
edker
atoder
ma

• Al
opeci
a,
Leoni
nef
aci
es,
Hepat
ospl
eenomegal
y

• Epi
dermotr
opi
sm by 
neoplast
ic 
CD4+ l
ymphocy
tes 
wit
hthefor
mat
ionof
Paut
ri
er'
smicr
oabscessesisthehal
lmarksi
gnofthedi
sease

• Erythr
oder
maassociat
edwi
thl
ymphomashowcl
assi
cal
feat
ures&f
ail
sto
respondtost
eroi
ds.

• Di
seasewi
l
lpr
ogr
ess,
Year
sdi
ff
icul
ttopi
npoi
ntet
iol
ogy
.

• Sezar
ycel
l
s.

HPE

• Nucl
earat
ypi
ainl
ymphocy
tes

• Cl
ust
eri
ngofat
ypi
cal
cel
l
swi
thi
nepi
der
mis,
Oft
enl
i
chenoi
dinf
il
tr
ate

Pi
tyr
iasi
srubr
api
lar
is

• I
nit
ial
lesi
on:
sebor
rhei
cder
mat
it
is-
li
keer
upt
ionoft
hescal
p

• Cephal
ocaudal
progr
essi
on

• Wor
seni
ngpostsunexposur
e

• Gener
ali
zedsal
mon-
col
oredsl
i
ght
lyi
nfi
l
trat
eder
ythema

• I
slandsofspar
ing

• Pal
mopl
ant
arker
atoder
ma,
oft
enor
ange

• Fol
li
cul
arpi
nkpapul
esofel
bows,
wri
sts,
dor
sal
finger
sori
nski
nspar
edf
rom
ED
 • Appear
anceofer
ythemagy
rat
um-
li
kel
esi
onsaser
ythr
oder
mai
mpr
oves

HPE

• Psor
iasi
for
m Epi
der
mal
hyper
plasi
a

• Hor
izont
alandv
ert
ical
alt
ernat
ingor
thoker
atosi
sandPar
aker
atosi
s

• Fol
l
icul
arpl
ugswi
th“
shoul
derpar
aker
atosi
s”

Der
mat
it
is(
non-
atopi
c),
incl
udi
ngcont
actandst
asi
swi
thaut
osensi
ti
zat
ion

• Pr
e-exi
sti
ngl
ocal
i
zeddi
sease

• Di
str
ibut
ionofi
nit
ial
lesi
ons

• Occupat
ionandhobbi
es

• Pat
cht
est
ing

• Rev
iewor
almedi
cat
ions(
syst
emi
ccont
actder
mat
it
is)

HPE-Var
iabl
espongi
osi
s

Par
aneopl
ast
icer
ythr
oder
ma

• Fi
nescal
i
ng,
Mel
anoer
ythr
oder
ma,
Cachexi
a

• Ly
mphopr
oli
fer
ati
vedi
sor
der
s,i
ncl
udi
ngot
hert
ypesofT-
cel
ll
ymphoma

• sol
i
d-or
ganmal
i
gnanci
es,
seenusual
l
yatl
atest
age

HPE-NONSPECI
FIC

BULLOUSDERMATOSES

Pemphi
gusf
oli
aceus

• Pr
e-exi
sti
ngl
esi
ons,
oft
enonuppert
runk

• I
mpet
igo-
li
keer
osi
onsandv
esi
cles

• Moi
stscal
e-cr
ust
;cor
nfl
akescal
e

• •
HPE-
Acant
hol
ysi
s,super
fi
cial
epi
der
mis

• DI
F,i
nter
cel
l
ularI
gG

• I
IF,
int
ercel
l
ular

Bul
louspemphi
goi
d

• Ur
ti
car
ial
plaques
 • Tensebul
l
a

• El
der
lypat
ient
s

• HPE-Subepi
der
mal
bli
ster

• Eosi
nophi
l
s

• DI
F,BMZC3andI
gG

• I
IF,
BMZ

Par
aneopl
ast
icpemphi
gus

• Mucosal
erosi
onsandhemor
rhagi
ccr
ust
s

• Er
ythemamul
ti
for
me-
li
kel
esi
ons

• Mucocut
aneousl
i
chenoi
dlesi
ons

HPE

• I
nter
faceder
mat
it
iswi
thnecr
oti
cker
ati
nocy
tes

• Focal
acant
hol
ysi
s

• DI
F,i
nter
cel
l
ularandBMZI
gG

• I
IF,
int
ercel
l
ularandBMZ(
+II
Fratbl
adder
)

Papul
oer
ythr
oder
maofOf
uji-Rar
e

• Widespread,
prur
it
ic,
flat
-t
oppedr
ed–br
ownpapul
est
hatcanbecome
confl
uent

• Spar
ingofski
nfol
ds(
“deck-
chai
r”si
gn)

• Fav
orsel
der
lymen

HPE-Denseper
ivascul
ari
nfi
l
trat
eofl
ymphocy
tesandeosi
nophi
l
sinuppert
omi
dder
mis

Chr
oni
cact
ini
cder
mat
it
is

• I
nit
ial
lesi
onsi
nphot
odi
str
ibut
ion

• Li
chenoi
dinf
il
tr
ateofl
ymphocy
tes

• Exocy
tosi
sPossi
bleofl
ymphocy
te

• Nucl
earpl
eomor
phi
sm

• Dr
ughi
stor
y
 • UVA,
UVBandv
isi
blel
i
ghtphot
otest
ing

• Phot
opat
cht
est
ing

Acut
egr
aft
-ver
sus-
hostdi
sease

• Hi
stor
yofbonemar
rowt
ranspl
ant
ati
onorbl
oodt
ransf
usi
on

• I
nit
ial
lesi
on:
pal
marer
ythema

• Pr
ogr
essi
ont
otoxi
cepi
der
mal
necr
oly
sis

Der
mat
omy
osi
ti
s

• Got
tr
onpapul
es

• Hel
i
otr
opesi
gn

• Poi
ki
loder
ma

• Per
iungual
tel
angi
ect
asi
a

• Muscl
eweakness

Nor
wegi
anscabi
es

• Downsy
ndr
omeornur
singhomepat
ient

• Bur
rows

• Massi
vel
ythi
ckenednai
l
s

• Ker
atoder
ma

HPE

• Super
fi
cial
anddeepper
ivascul
arandi
nter
sti
ti
ali
nfl
ammat
ion

• Manyeosi
nophi
l
s

• Fr
equentspongi
osi
s

• St
rat
um cor
neum wi
thmi
teorexcr
etaandcr
ust
ingpossi
ble

• Scr
api
ngofbur
rowst
oident
if
yami
teorexcr
eta

Compl
icat
ionsofer
ythr
oder
ma

• Shor
tnessofbr
eat
h–hi
ghoutputcar
diacf
ail
ure

• I
ncr
easedt
hir
st,
oli
gur
ia–r
enal
inv
/dehy
drat
ion

• Di
arr
hea–der
mat
opat
hicent
eropat
hy

• Cr
ams–dy
sel
ect
rol
emi
a
 • Cough–pneumoni
ti
s

• Pedal
edemaandcal
fpai
n-DVT

Pat
homechani
sms

Al
ter
edhemody
nami
cs

per
sist
enti
nfl
amat
ionofski
n

per
ipher
alv
asodi
l
atat
ion

i
ncr
easedcut
aneousbl
oodf
low

cl
i
nical
l
yer
ythemaandedema

Inadditi
on,t
herei
saincr
easedbloodvolumeandcardiacoutput,
whichmayjeopardi
zean
alreadycompromi
sedcardi
ovascul
arsystem(
hyper
tension,
ischemicorv
ascularhear
t
disease)

hi
ghoutputcar
diacf
ail
ure

Al
ter
edt
her
mor
egul
ati
on

Damagedker
ati
nocy
tes

I
ntr
eleuki
n1

Fev
eri
ntheabsenceofi
nfect
ion

Underl
yingcondi
ti
onsli
kepsor
iasi
s,ski
nisal
readyhy
po/
anhi
drot
ic,
duet
oint
raepi
der
mal
occl
usionofsweatducts

Hy
per
ther
mia

Met
abol
icabnor
mal
it
ies:

• Asacompensat
orymechani
sm f
ort
otal
heatl
oss,
ther
eisar
iseoft
hebasal
met
abol
icr
ate(BMR).

• Ahyper
gly
cemicstateandgl
ycosur
iaar
ecommoninthesepati
entsand
r
esul
tfr
om associ
atedpancr
eati
ti
sanddecr
easedi
nsul
insecr
eti
on,rel
ati
ve
i
nsul
inr
esist
ance,
stress,
andinf
ecti
on.

• Al
ter
edglucosemetabol
i
sm enhancescal
ori
clossbydepl
eti
ngt
issuepr
otei
n
asanenergysour
ce.

• Shiv
ering(highgr
ademuscul
aract
ivi
ty)
,asacompensat
orymechani
sm f
or
hypother
mi a,i
sal
sohi
ghl
yenergyconsuming.

Fl
uidandel
ect
rol
ytei
mbal
ance:
.
Theusualtot
alwaterl
osst
hroughtheski
nis400ml/day (600-
1000ml
/dayi
ntemper
ate
cl
imates)
.erythr
oderma-impair
edbarri
erf
uncti
onoftheski n
i
ncr
easedt
ransepi
der
mal
wat
erl
oss(
TEWL)

f
lui
drepl
acementi
snotadequat
e

r
educt
ioni
nthei
ntr
avascul
arv
olume

f
ormat
ionofhy
per
osmol
arur
ine.

dehy
drat
ionanddecr
easedur
inar
yout
put
.

Thi
sisassoci
atedwithel
ectrol
yteimbal
ance(
lowNa+andhi
ghK+)
,andr
aisedser
um l
evel
s
ofur
eaandcreati
nine(pr
erenaluremi
a).

Lossofnut
ri
ent
s

• Pr
inci
palnut
ri
ent
slostar
epr
otei
nandi
ron.

• maincausesofhy poprotei
nemiaareconti
nuouslossthroughshedscales,
i
ncreasedBMR, decreasedhepati
csynthesi
s,der
mat ogenicent
eropat
hy
l
eadingtoprot
einloss

• Theri
seinBMRseenamongpati
ent
swi
ther
ythr
oder
maoccur
satt
hecostof
cat
aboli
sm oft
issuepr
otei
n.

• Al
ter
ati
oni
ngl
ucosemet
abol
i
sm enhancesf
urt
herdepl
eti
onoft
issuepr
otei
n.

• Thecumulat
iveeff
ectofthesef
act
orsisanegat
iveni
tr
ogenbal
ance,
i
ncreasedur
inar
ynitr
ogenandhypoal
buminemia

• I
nadditiont
othel
ossthrought
heshedski
n,t
her
eisi
mpai
redabsor
pti
onand
ut
il
izat
ionofir
onandv
itaminB12.

• Ani ncr
easedcel
lul
arturnov
errat
egi
vesr
iset
orel
ati
vef
olat
edef
ici
ency
.Al
l
thesefactor
scontr
ibutetoanemi
a

• Lossofpr
otei
nperday–200g

• Lossofscal
es

9gm/
m2/
dayi
npsor
iasi
s

6gm/
m2/
dayi
neczemaandot
hercauses

Pul
monar
ycompl
icat
ions:

• Sev
erepul
monar
yedema(
secondar
ytocapi
l
lar
yleaksy
ndr
ome)

• adul
trespi
rat
orydi
str
esssy
ndr
ome(
ARDS)

• Over
zeal
ouscorr
ect
ionofhy
pov
olemi
afr
equent
lygi
vesr
iset
oov
ert
pul
monaryedema.

Per
ipher
aledema-Commoni
nlongst
andi
ngcases.
Pr
obabl
ecausesar
e:

• Incr
easedcapi
l
lar
yleakageandconsequentshi
ftoff
lui
dtoext
rav
ascul
ar
spaces

• Signi
fi
cantl
yincr
easedlevel
sofcircul
ati
ngvascul
arpermeabi
li
tyf
actor
(VPF)/vascul
arendothel
i
algrowthfact
or(VEGF)arefoundi
npsori
asis,
eczemaandot hercausesoferyt
hroder
ma

• Hy
poal
bumi
nemi
a

• Associ
atedcar
diacf
ail
ure

• I
nfl
ammat
ionr
esul
ti
ngf
rom t
hepr
imar
yski
ndi
sease

• VPFandVEGFar emulti
funct
ional
cytoki
nesli
ber
atedbyl
esi
onal
kerat
inocyteswhichinducedermalcapil
l
arypr
oli
fer
ati
onandenhance
microvascularpermeabi
lit
y.

Causesofmor
tal
it
y:

• Hi
ghout
putcar
diacf
ail
ure·

• Adul
trespi
rat
orydi
str
esssy
ndr
ome

• Capi
l
lar
yleaksy
ndr
ome

• Sepsi
s

Pr
ognosi
s

• Poori
nelder
lypat
ient
swi
thsy
stemi
cinv
olv
ementandmet
abol
i
c
di
stur
bances

Mal
i
gnancyassoci
ated.

• Good–dr
ugi
nduced

Poi
ntst
oremember

• Pati
entswit
hext
ensi
veski
nlesi
onsmaybef
ebr
il
eev
eni
nabsenceof
i
nfecti
on.

• Suddenonsethypot
her
miainar el
ati
vel
yst
abi
l
izedpat
ientmaybea
premoni
tor
ysignofsept
icshock.

• Apulserateof120ormor
e/minut
e,ev
eninpr
esenceofpreci
pit
ati
ngf
act
ors
l
ikesept
icemiaandfev
eri
ndi
cat
esanegat
ivef
lui
dbalance.

• Anor
mal
bloodpr
essur
emaybemi
sleadi
ngi
nsuchacase.

• I
ncr
easedr
espi
rat
oryr
atemaybet
hef
ir
stsi
gnofhy
poxi
aresul
ti
ngf
rom
 pneumoni
a/pul
monar
yedema.

• Areducti
oninur
inev
olumemaybeanear
lyi
ndi
cat
orofhy
pov
olemi
aor
sept
icemia.

• Analter
edsensor
ium,
int
hef
orm ofanxi
etyorconf
usi
onmaybet
hef
ir
stsi
gn
ofsepsi
s.

• Overzeal
ousflui
dcorr
ectionmaypreci
pit
atehighoutputhear
tfai
l
urein
pat
ientswit
hcompr omisedcar
diov
ascularsystem,ormaygiveri
seto
pul
monar yedema.

• Thoughserum concent
rat
ionr
epr
esent
sanor
mal
val
ue,
occul
thy
pokal
emi
a
mayexist
.

Ev
aluat
ionofcase

• Hi
stor
y

• Onset,
dur
ati
on

• Acut
e–dr
ugi
nduced,
PRP,
ster
oidwi
thdr
awl
inps

• Pr
uri
tus -execessi
vei
nadorl
yphoma

• f
orcompl
i
cat
ions

PROGRESSI
ON

Cephal
ocaudal
spr
ead–PRP

ORI
GINOFRASH

• Fl
exur
es–At
opi
cs

• Fl
exur
al+sebor
rhoei
c–SD

• Head,
neckandext
remi
ti
es–CD

• Gai
terr
egi
on–st
asi
sder
mat
it
is

PASTHI
STORY

• Seasonal
var
iat
ion

• H/
Oat
opyi
npat
ientandf
ami
l
y

• I
cthyscal
ylesi
onov
erel
bowandknee

• Changei
nresi
dence,
wor
kpl
ace

• H/
Ophot
osensi
ti
vi
ty,
proxi
mal
muscl
eweakness,
oral
ulcer
s

• Joi
ntpai
n
 • Wei
ghtl
oss/
appet
it
e

• Fl
uidf
il
ledl
esi
ons

• Dr
ughi
stor
y-i
ncl
udet
opi
cal
medi
cat
ions,
drugwi
thdr
awl
.

• Faml
i
lyhi
stor
yofat
opy,
ski
ndi
seasesl
i
kepsor
iasi
s,
PRPordr
ugal
l
ergi
es

Exami
nat
ion

Gener
al

• TPR/
BP

• hemody
nami
cst
atus

• Pal
l
or,
i
cter
us,
cynosi
s,
cl
ubbi
ng

Pedaledema

• Renal
inv
olment

• Hy
poal
bumi
n

• Vasodi
l
atat
ion

Ly
mphnodes

• Formal
i
gnaci
es

• Der
mat
opat
hicl
ymphadenopat
hyi
neczemas

• HI
V/secsy
phi
l
is

Sy
stemi
c

CNS,
CVS,
RS

P/
A

• Hepat
omegal
y

• Mal
i
gnancy
,dr
uget
iol
ogy

• Spl
enomegal
y

• Mal
i
gnancy

Cut
aneousexami
nat
ion

• Di
str
ibut
ion

• Lookf
orspar
edar
eas

• Face–psor
iasi
smost
ly
 • Di
aperar
easandf
inger
s–AD

• I
slandsofspar
ingi
nPRP

• Spar
ingoff
olds–CTCL(
deckchai
rsi
gn)

Col
our

• Deeppur
pul
e-r
ed–CTCL

• Or
ange–PRP

Bl
ist
ers,
rawar
eas,
crust
ing-Secondar
yinf
ect
ions,
aut
oimmunebl
i
ster
ingdi
sor
der
s(BP,
PF)

Tender
nessov
erski
n,Ni
kol
sky-
SSSS,
P.f
oli
aceous

Obser
vescal
es

Ty
pes –Mi
caceous–psor
iasi
s

• Fi
ne–AD/
der
mat
ophy
tei
nf

• Gr
easy–SD

• Cor
nfl
akel
i
kescal
es–PF

• Post
ery
themadesquamat
ion–dr
ugr
eact
ions/
bact
eri
ali
nfect
ions

• Pot
ato–peel
scal
i
ngi
nSSSS

Si
zeofscal
es-l
arge-
acut
e,smal
l
-chr
oni
c

Pal
msandsol
es-
PRPandpsor
iasi
s

Fi
ssur
edker
atoder
ma–CTCL

Mucosa-Or
al/
geni
tal
ulcer
s,Gl
ossi
ti
s

Nai
lchanges

• Def
luv
ium ungui
um(
hypopr
oti
enemi
a.l
ossofsul
phuri
nscal
es)

• Nai
lchangesofpsor
iasi
s/PRP

• Beau’
sli
ne–chr
oni
cer
ythr
oder
ma

• Shor
eli
nes

Hai
r–t
elogenef
fl
uvi
um

Ey
e–ect
ropi
on,
blephar
it
is,
epi
phor
a
APPROACH
WI
THSI
RS;

St
abi
li
zat
ionandski
ncar
e

• Room –war
mtemp,
70%humi
dit
yvi
ahumi
dif
ier

• Vi
tal
s,
I/
Ochar
ti
ng

• Hy
drat
e,cor
rectel
ect
rol
ytei
mbal
ance

• Pr
otei
n,
zi
nc,
i
ronandf
oli
caci
dsuppl
ement
ati
on

• Lukewar
m compr
esseswi
thchl
orhexi
midebat
hs

• Bl
andemol
l
ient
s

• Ant
ihi
stami
nesf
orpr
uti
tus

• Pedal
edema-el
ast
ocr
epebandage,
iff
ail
slasi
x

I
nvest
igat
ionsi
nSI
RS

• ESR/
CRP/
procal
cit
oni
n,Bl
oodcul
tur
e,Chestxr
ay

• ABG

I
nvest
igat
ionst
olookf
orcompl
icat
ions
 • CBC-Lowhb

Anaemi
aofchr
oni
cdi
sease

I
ncr
easel
ossofbl
oodf
rom ski
nor

Gutmal
absor
pti
on

• El
ect
rol
ytes

• Ski
nswabofnost
ri
ls/
areasofseci
mpet
igi
nizat
ion(
pust
ularcr
ust
s)oft
heski
n

• Tzancksmear

I
mmunobul
l
ousds,
kaposi
var
iecel
l
ifor
m er
upt
ions

Bl
oodbor
nev
irusscr
een(
CD4count
sifHI
Vposi
ti
ve)

• Or
gandy
sfunct
ion

• LFT-hepat
it
is

• RFT,
uri
neanal
ysi
s-nephr
it
is

• CXR-pneumoni
ti
s

I
nvf
orcause

Er
ythr
oder
mawi
thoutSI
RS -Ski
ncar
e

I
nvest
igat
eforcause;

CBCwi
thPS

• Hi
ghTC-i
nfect
ions,
abnor
mal
cel
l
sindi
cat
eleukemi
ccondi
ti
ons.

• Eosi
nphi
l
ia–dr
ugr
eact
ions,
ACD,
bul
l
ouspemphi
goi
d,i
diopat
hichy
per
eosi
nophi
l
ia
syd.

• LowTC-
SLE,
immunodef
ici
enci
es

• KOHswab

• I
gE-AD,
bul
l
ouspemphi
goi
d

• Ur
ineanal
ysi
s-eosi
nophi
l
sindr
ugr
eact
ions

• ANA

• LDH

• Bi
opsyf
rom mul
ti
pleski
nsi
tesandLN

• DI
FinI
BDandCTD
 • Mal
i
gnancyscr
eeni
ng

• VDRL/
HIV

FLUI
DMANAGEMENT

• Goal-Topr
eventhy
pov
olemi
cshock

• Ri
ngerl
act
ate(
RL)
ornor
mal
sal
i
newi
th5%gl
ucose.

• Mai
ntai
nance-RLwi
th5%gl
ucoseorhal
fnor
mal
sal
i
newi
th5%gl
ucose

• Par
klandf
ormul
a-4ml
×BSA(
%)×wei
ght(
kg)

• Gi
vehal
foft
hesol
uti
onf
or,
FIRST8HOURS

• Gi
vet
heot
her
,hal
foft
hesol
uti
onf
or,
NEXT16HOURS

• Or
alsuppl
i
ment
ati
onmayst
artaf
ter48hour
s.

Concl
usi
on

Er
ythr
oder
mai
sacl
i
nical
mani
fest
ati
onsofav
ari
etyofunder
lyi
ngdi
seases.

Pot
ent
ial
riskf
ormor
bit
yandmor
tal
i
ty,
hospi
tal
i
zat
ioni
sof
tenr
equi
red.

I
nit
ial
managementi
ncl
udesr
emov
inganypot
ent
ial
off
endi
ngagent
.

I
ndoubtconsi
derTcel
llcl
onal
i
tystudi
esandfl
owcy t
omet
ryf
orsezar
ysy
ndr
ome,
whi
chcan
mimi
cotherder
matosesonpathol
ogyandcli
nical
exam