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Dermatology emergencies grand rounds
1. All information about the condition, medical history
or treatment of patients disclosed at this clinical
meeting is absolutely confidential.
The Health Information Privacy Code (1994)
applies to all present whether or not they are
employees of, visitors to, or studying at the
Canterbury District Health Board or the
University of Otago, Christchurch.
REMINDER-cell phones to vibrate mode please
4. Disclaimer
ďNot exhaustive (SSSS, immunobullous, Kawasakiâs
disesase)
ďApproach to clinical scenarios
ďConditions discussed are emergencies because they
can lead to âacute skin failureâ â i.e. Loss of
thermoregulatory/metabolic/infection control
mechanisms of skin
7. Widespread pustules
ďGeneralised pustular psoriasis
ďAcute generalised exanthematous pustulosis
ďFolliculitis-bacterial/viral/eosinophilic/pityrosporum
ďDisseminated HSV (rarely)
ďNeutrophilic dermatoses-Behcetâs/Sweetâs/PG
ďIgA pemphigus
ď(Exanthematous DE â often have a few pustules of
follicular origin)
9. GPP
ďAcute/subacte/chronic-superimposed on plaque type disease or
de novo after developing atypical, acral or flexural disease in
later life
ďAcute form is an âemergencyâ= von Zumbusch variant
ďClinical features: Warning signs â burning, tenderness,driness
ďAbrupt onset high fever and severe malaise
ďPre-existing plaques become fiery and superimposed pustules
ďSheets of erythema and waves of pustulation spread to
involved previously normal skin, esp. Flexures and genitals
ďRemission days-weeks +/- erythroderma+/-relapse
11. Complications of Erythroderma
âTHE I NET +metabolicâ
ďThermoregulation/Thrombosis
ďHaemodynamic- renal
perfusion/CHF/pneumonia/oedema
ďEctropion
ďInfection - cutaneous and respiratory
ďNutrition ( albumin)/nails/nodes
ďEnteropathy - Fe/B12/folate/protein/fat
ďTelogen effluvium
ďMetabolic â electrolyte imbalance
12. Complications of acute GPP
ďâTHE I NET Mâ
ďLow â albumin, calcium
ďCholestatic jaundice
ďDVT
ďSecondary Staph. aureus infection
ďInflammatory polyarthritis
ďAmyloidosis (rare)
ďObstetric complications
ďAcute telogen effluvium
13. Management acute GPP
ďTreatment:
ďWithdraw/treat provocative factors
ďAdmit to hospital
ďStrict bed rest
ďThromboprophylaxis +thermoregulation
(hypothermia)
ďFluid and nutritional support/electrolyte s
ďAnalgesia and antihistamines
14. Management acute GPP
ďTopical therapy: Bland emollients/wet
dressings/mild-moderate potency topical steroids.
ďTar and dithranol are contraindicated
ďSystemic therapy: Most require (difficult in
pregnancy)
ďAcitretin = treatment of choice
ďMTX/CyA/TNF-Îą blockers
ďOral steroids only when urgent control of metabolic
complications necessary/consider in pregnant
patients
15.
16. Acute generalised exanthematous
pustulosis (AGEP)
ď Acute febrile pustular eruption
ď > 90% drug induced (other causes â HS to mercury, enteroviral
infection)
ď Short time between drug and eruption <2- 4/7
ď Drugs causing AGEP: âBAD FACEâ-mostly penicillins and macrolides
ď Bactrim
ď Antibiotics and
antifungals(Vanc/Penicillins/Cephalosporins/Macrolides/terbinafine/i
traconazole)
ď Diltiazem
ď Frusemide
ď Allopurinol/antimalarials
ď Cimetidine
ď Epileptics
18. AGEP â Clinical Features
ďHigh fever (usu. Onset same day as rash)
ďNumerous small, primarily non-follicular, sterile
pustules arising within large areas of oedematous
erythema +/- burning, pruritus
ďLesions start face and flexures, then disseminate over
a few hours
19. Other features: facial & acral oedema, erythema multiforme-llike lesions,
vesicles, bullae, purpura, mucosal in 50%
20. Face, flexures, trunk, upper limbs
Lesions last 1-2 weeks; resolve with
superficial desquamation
21. AGEP - Treatment
ďStop the offending drug
ďSupportive measures â rest/bland emollients/topical
corticosteroids/occasionally prednisolone
26. Treatment- DRESS
ďStop the drug!
ďConsult as per investigations for organ involvement
ďCorticosteroids first-line (good for skin, heart and
lungs, but less useful to treat renal and liver disease)
ďPNL may be need to be tapered over many months to
avoid relapse
ďEmollients/antihistamines/TCS/wet dressings
28. Erythroderma
ďPresence of erythema and scaling involving more
than 90% of skin surface (can be caused by any
inflammatory skin condition)
ďPrimary: erythema (often initially on trunk) extends
within few days to weeks to involve whole skin
surface. Followed by scaling
ďSecondary: generalisation of a preceding localized
skin disease (e.g. psoriasis, atopic eczema)
ďAcute vs chronic
39. Erythroderma- Investigations
ďFor associated conditions:
ďIgE and Eâ°âs/patch/photo-patch
ďFBC+film/TCR-GR/Lâ° subsets/LDH/Sezary cell count
ďLymph node/BMBx
ďCXR/CT chest/abdo/pelvis
ďImmunohistochemistry on skin biopsy CD3/4/5/7 +/- CD
30+
ďSPE
ďESR
ďCa-serum/urine
ďANA/ENA/Câ/dsDNA/RhF
ďPre-treatment investigations â QF-gold/Hep etc.
40. Erythroderma - Treatment
ďAdmit to hospital (if acute/unwell)
ďManagement of fluid balance and temperature
ďReview medications (cease non-essential)
ďTopical (care re impaired barrier)
ďEmollients, +/- mild/mod topical steroids
ďWet dressings
41. Erythroderma - Treatment
ďTreat infection
ďAntihistamines
ďSystemic steroids in some (not if ?psoriasis)
ďThromboprophylaxis
ďReferrals â Nutrition/Cardiology
ďTreat the underlying disease!
42. Erythema multiforme-how do you tell that
this isnât a true emergency? (no risk of progression to TEN)
ďSelf-limited, but potentially recurrent disease
ďAbrupt onset symmetrical, fixed red papules, some of
which evolve into typical/atypical papular target lesions
ďTypical targets- at least 3 different zones
ďAtypical â only 2 different zones and poorly-defined
border
ďTarget lesions favour acrofacial sites; extremities and face
ďPainful/pruritic
ďTargets may blister
ď+/- mucous membranes
43. Classic targets / iris â triphasic
1) Central purple/ dusky area
2) White oedematous
concentric rim
3) Red halo
Dusky centres
44. EM minor vs major
EM minor EM major
ďTypical +/- atypical papular ďTypical > atypical targets
targets ďSevere mucosal and systemic
ďLittle or no mucosal
features
involvement
ďNo systemic symptoms
ďIn all EM, majority lesions
will develop within 24 hr (all
by 72 hr)
ďDuration episode approx.
2/52
45.
46.
47.
48. EM/SJS/TEN
EM SJS TEN
Rash Typical Atypical targets, blisters widespread
targets SJS <10%; TEN > 30%
Acrofacial + Mortality 5% SJS/30% TEN
limbs
Mucous Absent/ Severe
membrane Mild (unless
major)
Drug HSV >> drug Anticonvulsants, sulfonamides,
allopurinol, NSAID, b-lactams
50. ďUrticaria â ITCHY!
ďErythema multiforme
ď Central zone is normal skin
ď Central zone of epidermal
ď Lesions are transient, lasting
damage (dusky, bullous,
several hours crusted)
ď New lesions appear daily ď Lesions 'fixed' for at least 7
ď Associated with oedema hand days
and feet (angioedema) ď All lesions appear within
first 72 hours
ď No oedema
52. SJS/TEN
⢠Rare , acute life-threatening mucocutaneous diseases
⢠Overlapping features-both T-cell-mediated
⢠Extensive keratinocyte cell death â separation of skin
at DEJ
⢠Keratinocyte death via apoptosis â mediated by
interaction of the death receptor-ligand pair Fas-FasL
⢠Same precipitants-almost always DRUGS!!!
⢠The more widespread, the more likely drug cause (SJS
50% drug; TEN 90%)
53. Pathogenesis
ďGenetic susceptibility HLA-B12 â/HLA-B*5701, HLA-DR7
ďFailure to detoxify reactive +HLA-DQ3 = 100% predictive TEN with abacavir
intermediate drug
metabolites
ďImmune response to
antigenic complex
ďInteraction between Fas
(CD95) and its ligand on
epidermal cells triggers
apoptosis pathways and
cell death
54. SJS and TEN
⢠Spectrum of severity
â SJS < 10% epidermal detachment
⢠⼠2 mucosal sites
â SJS / TEN overlap 10-30%
â Toxic epidermal necrolysis >30%
⢠Large areas denuded skin
⢠TEN: compared to SJS: extensive confluence, large areas of denuded
skin; poorly delineated red plaques;
⢠At risk:
â Slow acetylators
â HIV (1000x)
â Lymphoma
â SLE
56. TEN
ďThe more widespread, the more likely drug cause (SJS 50%
drug; TEN 90%). Other causes=infections and immunisations
ďDrugs causing TEN: âLOV THE SANDâ
Lamisil +other antifungals
Omeprazole
Vancomycin
TB drugs
HIV drugs (esp. Nevirapine +abacavir)/herbs
Epileptics (phenytoin/CBZ/lamotrigine)
Sulphas (Bactrim,sulphasalazine), statins
Allopurinol (very common)/antibiotics (penicillins, Bactrim)
NSAIDS
Dapsone
57. SJS /TEN clinical features
⢠1-3 weeks after exposure to drug
⢠Prodrome (1-3 days)â malaise, fever, pharyngitis, eye
discomfort
⢠Skin lesions: Usually first on trunk, then neck, face and
proximal upper extremities
⢠Palms +soles may be involved early
⢠Erythema and erosions of oral, ocular, genital mucosae in
>90%
⢠Respiratory tract epithelium involved in 25%
⢠GIT mucosal erosions
⢠Skin and mucosal erosions tender and very painful
⢠Systemic manifestations: fever, LN, hepatitis, cytopenias
(neutropenia, lymphopenia,thromobcytopenia= poor
prognosis)
60. As necrosis becomes full-thickness, dusky âred
macular lesions become grey (hours-days)
61. Necrotic epidermis then detaches, fluid fills space
b/t epidermis and dermis flaccid blisters
which break easily Tense blisters usu. only on
palmoplantar surfaces
69. Important DDxâs for SJS/TEN
⢠Paraneoplastic pemphigus/pemphigus vulgaris/ bullous
pemphigoid(including drug-induced) â DIF/IDIF
⢠Linear IgA disease (+drug-induced)-DIF/histology
⢠Bullous lupus erythematosus - ANA/DIF
⢠Stage IV acute GVHD-evolution
⢠Kawasakiâs disease (children)
⢠Staphylococcal scalded skin â (frozen section/H+E)
⢠Acute generalised exanthematous pustulosis-self-limiting when cease drug
⢠SEE FEB. 2007 JAAD CME PAPER
70. Investigations in SJS/TEN
ďAs for erythroderma
ďCXR/UEC/LFTâs/Coags for systemic invlt
ďFBC â prognosis and evidence infection
ďSCORETEN â Day 1 and Day 3
ďBiopsy â H+E/DIF +/- frozen section
ďIndirect immunofluorescence â exclude PNP/PV
ďANA/ENA/dsDNA (before give IVIg)
ďRegular cultures â skin/blood/mucous membranes
ďViral swabs
ďIgA levels (before IVIg)
71. Early lesion: apoptotic keratinocytes Early lesion: separation of epidermis from
dermis; full thickness necrosis +bulla
formation; variable density dermal
mononuclear infiltrate (mostly T Lâ)
72. Management SJS/TEN
ďStop causative drug and all non-life-sustaining drugs
ďAdmit to hospital
ďRapid initiation-
ďa) Supportive
ďb)Specific management â controversial and evidence
is still evolving
73. Management SJS/TEN-Supportive
ďAdmit Burns Unit/ICU
ďCorrect/monitor fluid and electrolyte balance
ďNutrition â refer â replace calories/protein/etc
ďSurveillance for infection- regular swabs mouth, eyes,
skin, sputum (treat based on culture results and when
signs of sepsis â NOT PROPHYLACTICALLY)
ďAnalgesia â Pain team review
ďEye care â Consult Ophthalmology-lubricant
drops/steroid drops/chlorsig
ďUrology/Gynae â IDC/manual exam or dermeze tampons
ďMouth care â PMMW/xylocaine viscus 2%/Diprosone OV
ung/Daktarin oral gel/white soft paraffin lips
74. Management SJS/TEN-Supportive
ďPhysio. to prevent contractures and respiratory
ďSkin care â gentle handling/no tapes on skin/air
mattress/non-adherent dressings (Bactigras and
Acticoat)/biologic skin equivalents reported
ďCare to avoid pressure areas
ďThromboprophylaxis
ďMedic Alert Bracelet/ADR notification/counsel relatives
ďProton pump inhibitors for GIT prophylaxis
75. Management SJS/TEN-
Specific/Adjunctive
⢠CONTROVERSIAL! Complementary â apoptosis is
rapid + irreverisble once triggered so must be early (1st
4 days)
⢠Corticosteroids â deleterious effect in small studies/
possible benefit recent studies? Poor outcomes 2â
inadequate doses? (pulse dexa 1.5mg/kg/day for 3/7)
⢠CyA - ?anti-apoptotic via âregulation NF-ÎşB (3-4mg/kg/day)
⢠Cyclophosphamide
⢠Infliximab
⢠Plasmapheresis +/- IVIg (remove drug/metabolites/cytokines)
⢠IVIG high dose 2-4 g/kg
76. IVIG
IVIG â autoantibodies
against Fas receptor block
Fas-FasL binding and
thereby prevent (in vitro)
apoptosis
1g/kg for 3 days (total
3g/kg)
Miami group AAD 2011 â
use 1g/kg for 4/7
Survival ďą with every
1g/kg increment in dose
(OR = 4.2)
No prospective controlled
studies with sufficient
numbers. Doses varied in
previous studies
77. â˘HLA-B*1502 is strongly associated with carbamazepine-induced
TEN/SJS â reported in several independent studies
â˘Mostly observed in patients of South-East Asian descent
â˘This may also be seen in drugs with structural similarity to
CBZ in patients with HLA-B*1502
â˘HLA-B*5801 associated with allopurinol-induced TEN/SJS
â˘Screening for HLA-haplotypes ideal before using these drugs in
relevant patients
78.
79. Eczema Herpeticum/Kaposiâs varicelliform
eruption
ď Widespread cutaneous infection with a virus that normally
causes localised or mild vesicular eruptions â IN A PATIENT
WITH PRE-EXISTING SKIN DISEASE
ď Majority of cases are HSV-1 in atopic eczema = eczema
herpeticum
ď KVE = widespread infection with other viruses (coxsackie A16,
VZV)
ď Children and young adults usually (2 nd-3rd decade)
ď In eczema herpeticum, majority are primary infections, but can
occur with endogenous recurrent infection (herpes labialis)
ď Risk factors
ď Atopic dermatitis
ď Parental /close contacts herpes labialis
ď Other chronic skin disorders (pemphigus foliaceus, Darierâs, Hailey-
Hailey, MF, Sezary syndrome, ichthyosis vulgaris, CIE etc)
80. Clinical features
ďIncubation period 10 days
ďUnderlying skin disease
ďCrops of vesicles that rapidly become pustules (new
crops 5-7 days)
ďLesions begin in abnormal skin +/- generalise
ďPainful âpunched outâ erosions
ďSecondary staph infection
ďMonomorphic 2-3mm haemorrhagic crusts = clue
ďFever onset 2-3 d after eruption âlasts 4-5/7
ď+/- severe constitutional Sx
ďRegional lymphadenopathy
ďProgression to potentially fatal systemic infection
rarely
Change order â pustules, DRESS, TEN, erythroderma
Subtypes of pustular psoriasis â Localised (palmoplantar, acrodermatitis continua of Hallopeau) Generalised (acute, of pregnancy, infantile and juvenile, circinate, localised - not hands and feet)
Occ agran, low plt, coombs+ hâlytic an, apl an, autoimmune thyroiditis after 2m
Fine in atopic dermatitis or dermatophytosis, branny in seb derm, crusted in PF, thicker in psoriasis. Skin hot to touch
Waxy orange PPKD, follicular horny plugs elbows+ knees and dorsal fingers and toes, islands of sparing+PRP, crusted erosions and flaccid bullae in PF, pre-existing nail changes such as pits etc in psoriasis, horizontal ridging in AD
Lichenification and excoriations
Islands of sparing, follicular accentuation
Complications?
Due to some clinical similarity, EM/SJS/TEN all previously considered to be spectrum. New concept that EM is a distinct disease entity on several levels (clinical, prognostic, aetiologic)
Concentric zones of colour change. Usually greater than 100 lesions. Recurrences are usual
What is the most common cause of EM?
HSV may be facial or genital. Mycoplasma is another common cause.
These two entities are frequently confused
TEN: compared to SJS: extensive confluence, large areas of denuded skin; poorly delineated red plaques;
Exfoliation due to extensive death of Kâs mediated by interaction of the death receptor-ligand pair, Fas-Fas ligand on the surface of Kâs
Drugs started 14+ days prior to onset. Genetic differences in detoxification likely to be responsible.
Recent report TEN secondary to beta-blocker eyedrops
Skin is extremely painful
Prognosis is highly correlated with extent of skin detachment. Prognosis also correlates with SCORTEN in which these 7 parameters are given equal weight
Biopsy needed to differentiate all. Vancomycin can induce both TEN and LAD â mucosal involvement is rare in DI-LAD. BMT pts receive many meds known to cause TEN and mucositis following chemo-induced neutropenia is common â folliculocentric, begins acrally and spreads proximally/TEN begins centrally and spreads periph
Fluid loss is significant Increased energy expenditure so hight caloric intake needed
By day 6-10 of admission, most of the skin erosions will re-epithelialise
IVIg remains preferred treatment in our unit â anecdotally good experience/helps combat infection