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

2. Dermatology
Emergencies?!?
Really???
Dermatologist
Newbie? That’s
Greek for “not
real doctor”

3. Dr Katherine Armour
Consultant Dermatologist
Canterbury District Health

4. Disclaimer
Not exhaustive (SSSS, immunobullous, Kawasaki’s
disesase)
Approach to clinical scenarios
Conditions discussed are emergencies because they
can lead to “acute skin failure” – i.e. Loss of
thermoregulatory/metabolic/infection control
mechanisms of skin

5. Overview
Recognition and clinical features
Aetiology
Complications
Management

7. Widespread pustules
Generalised pustular psoriasis
Acute generalised exanthematous pustulosis
Folliculitis-bacterial/viral/eosinophilic/pityrosporum
Disseminated HSV (rarely)
Neutrophilic dermatoses-Behcet’s/Sweet’s/PG
IgA pemphigus
(Exanthematous DE – often have a few pustules of
follicular origin)

8. Acute
generalised
pustular
psoriasis

9. GPP
Acute/subacte/chronic-superimposed on plaque type disease or
de novo after developing atypical, acral or flexural disease in
later life
Acute form is an “emergency”= von Zumbusch variant
Clinical features: Warning signs – burning, tenderness,driness
Abrupt onset high fever and severe malaise
Pre-existing plaques become fiery and superimposed pustules
Sheets of erythema and waves of pustulation spread to
involved previously normal skin, esp. Flexures and genitals
Remission days-weeks +/- erythroderma+/-relapse

10. Triggers for acute GPP
Irritating topical therapy – tar/dithranol
Infection
Pregnancy
Hypocalcaemia
Infection
Drugs – salicylates, iodide, lithium, terbinafine
Withdrawal of systemic corticosteroids/potent
TCS/cyclosporin

11. Complications of Erythroderma
“THE I NET +metabolic”
Thermoregulation/Thrombosis
Haemodynamic- renal
perfusion/CHF/pneumonia/oedema
Ectropion
Infection - cutaneous and respiratory
Nutrition ( albumin)/nails/nodes
Enteropathy - Fe/B12/folate/protein/fat
Telogen effluvium
Metabolic – electrolyte imbalance

12. Complications of acute GPP
“THE I NET M”
Low – albumin, calcium
Cholestatic jaundice
DVT
Secondary Staph. aureus infection
Inflammatory polyarthritis
Amyloidosis (rare)
Obstetric complications
Acute telogen effluvium

13. Management acute GPP
Treatment:
Withdraw/treat provocative factors
Admit to hospital
Strict bed rest
Thromboprophylaxis +thermoregulation
(hypothermia)
Fluid and nutritional support/electrolyte s
Analgesia and antihistamines

14. Management acute GPP
Topical therapy: Bland emollients/wet
dressings/mild-moderate potency topical steroids.
Tar and dithranol are contraindicated
Systemic therapy: Most require (difficult in
pregnancy)
Acitretin = treatment of choice
MTX/CyA/TNF-α blockers
Oral steroids only when urgent control of metabolic
complications necessary/consider in pregnant
patients

16. Acute generalised exanthematous
pustulosis (AGEP)
 Acute febrile pustular eruption
 > 90% drug induced (other causes – HS to mercury, enteroviral
infection)
 Short time between drug and eruption <2- 4/7
 Drugs causing AGEP: “BAD FACE”-mostly penicillins and macrolides
 Bactrim
 Antibiotics and
antifungals(Vanc/Penicillins/Cephalosporins/Macrolides/terbinafine/i
traconazole)
 Diltiazem
 Frusemide
 Allopurinol/antimalarials
 Cimetidine
 Epileptics

17. Small non-
follicular
pustules

18. AGEP – Clinical Features
High fever (usu. Onset same day as rash)
Numerous small, primarily non-follicular, sterile
pustules arising within large areas of oedematous
erythema +/- burning, pruritus
Lesions 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
Stop the offending drug
Supportive measures – rest/bland emollients/topical
corticosteroids/occasionally prednisolone

22. • Fever Well
• LAD Mild eosinophilia
• Elevated LFTs, eosin, atypical
lymphocytes

23. DRESS
(Drug reaction with eosinophilia and systemic symptoms), aka drug
hypersensitivity syndrome
– Triad: fever, rash, internal organ involvement
(hepatitis, lymphadenopathy, nephritis, pneumonitis,
haematological, myocarditis, thyroiditis – atypical ↑L∅, ↑N,
↑eos)
– Onset 1-8 wks
– Prodrome: fever, malaise, pharyngitis
– Rash: morbilliform (usually), exfoliative, erythrodermic,
pustular, SJS, TEN
– Onset rash usually face/upper trunk +extrem.
– Features suggesting: facial oedema, eosinophilia (90%),
atypical lymphocytes (40%), deranged LFTs,
lymphadenopathy
– Mortality 8%
– Drug causes: “MATES”
Minocycline/allopurinol/ARV/terbinafine/epileptics/sulfas
incl. dapsone

24. DDx of DRESS
Other cutaneous drug reactions
Viral infections
Idiopathic hypereosinophilic syndrome
Lymphoma/pseudolymphoma

25. Investigations - DRESS
FBC – eosinophilia and atypical lymphocytosis
UEC )
Elevated hepatic enzymes ) close monitoring
CXR – pulmonary infiltrates
Biopsy for H+E – Dense superficial dermal
lymphocytic infiltrate +eosinophils/dermal
oedema+/-pseudolymphomatous (if chronic)

26. Treatment- DRESS
Stop the drug!
Consult as per investigations for organ involvement
Corticosteroids first-line (good for skin, heart and
lungs, but less useful to treat renal and liver disease)
PNL may be need to be tapered over many months to
avoid relapse
Emollients/antihistamines/TCS/wet dressings

27. ERYTHRODERMA

28. Erythroderma
Presence of erythema and scaling involving more
than 90% of skin surface (can be caused by any
inflammatory skin condition)
Primary: erythema (often initially on trunk) extends
within few days to weeks to involve whole skin
surface. Followed by scaling
Secondary: generalisation of a preceding localized
skin disease (e.g. psoriasis, atopic eczema)
Acute vs chronic

29. Causes of Erythroderma
Drug causes: DESIGN CHAMP
“Top 5” – “PEDLI”  Diuretics (frusemide +thiazides)
 Epileptics
Psoriasis  Sulfas
Eczema  Isoniazid (TB-RIPE)
Drug eruptions  Gold
 NSAIDS
Lymphoma
 Captopril/ACEI
Idiopathic  Homeopathy/Herbs
 Allopurinol/antibiotics
 Malarials
 Psychotics (anti) - lithium

30. Causes of Erythroderma-”PENCIILS
GID”
Psoriasis and variants - PRP/Seb. Derm/Reiter’s
Eczema-atopic/irritant/allergic/CAD
Neoplasia-lymphoma/CTCL/SS/leukaemia/solid organ
CTD – LE/DM/Sjogren’s
Infection – scabies/HIV/d’phyte(T.violaceum), SSSS, TSS
Immunobullous /acantholytic– PF/BP/Hailey-Hailey,
Darier’s
Lichen planus
 Sarcoidosis
Graft vs host disease

31. “PENCIILS GID”
Ichthyosiform erythrodermas – lamellar
ichthyosis/Netherton’s syndrome/CIE/BIE
Drugs- should improve within 2-6/52 off drug unless
DRESS

32. Erythroderma – clinical
features
Rapidly extending erythema (may be universal in
12-48 hrs – esp. rapid in primary eczema and
lymphoma)
Fever, shivering, malaise- hypo>hyperthermia
(low –reading thermometer)
Scale (fine/branny/large) –after 2-6 days
Pruritus (90%) + tightness of skin
Lichenification

33. Erythroderma – clinical
features
Lymphadenopathy-extent variable. Dermatopathic
(sl-mod. enlarged and rubbery) vs 2⁰ lymphoma
Weeks: Hair-diffuse, non-scarring alopecia (20%
chronic)
Nails (40%)– “shiny” , discoloured, subungal
hyperkeratosis, Beau’s lines, splinter hge’s
Multiple seborrhoiec keratoses
Oedema – lower legs/ankles
Clues – underlying skin disease

37. Complications of Erythroderma
“THE I NET +metabolic”
Thermoregulation/Thrombosis
Haemodynamic- renal
perfusion/CHF/pneumonia/oedema
Ectropion
Infection - cutaneous and respiratory
Nutrition ( albumin)/nails/nodes
Enteropathy - Fe/B12/folate/protein/fat
Telogen effluvium
Metabolic – electrolyte imbalance

38. Erythroderma- Investigations
Diagnosis: Biopsy – H+E/DIF (+/-)/TCR-GR
Skin scrapings
• Extent/Cx: Blood cultures if febrile
• FBC – eosinophilia/lymphocytosis
• UEC/Ca/Mg/PO4/LFTs/protein/albumin
• MSU
• Fe/B12/folate
• Skin swabs
• CXR-CHF/Ca/pneumonia
• ECG – if elderly

39. Erythroderma- Investigations
For associated conditions:
IgE and E⁰’s/patch/photo-patch
FBC+film/TCR-GR/L⁰ subsets/LDH/Sezary cell count
Lymph node/BMBx
CXR/CT chest/abdo/pelvis
Immunohistochemistry on skin biopsy CD3/4/5/7 +/- CD
30+
SPE
ESR
Ca-serum/urine
ANA/ENA/C’/dsDNA/RhF
Pre-treatment investigations – QF-gold/Hep etc.

40. Erythroderma - Treatment
Admit to hospital (if acute/unwell)
Management of fluid balance and temperature
Review medications (cease non-essential)
Topical (care re impaired barrier)
Emollients, +/- mild/mod topical steroids
Wet dressings

41. Erythroderma - Treatment
Treat infection
Antihistamines
Systemic steroids in some (not if ?psoriasis)
Thromboprophylaxis
Referrals – Nutrition/Cardiology
Treat the underlying disease!

42. Erythema multiforme-how do you tell that
this isn’t a true emergency? (no risk of progression to TEN)
Self-limited, but potentially recurrent disease
Abrupt onset symmetrical, fixed red papules, some of
which evolve into typical/atypical papular target lesions
Typical targets- at least 3 different zones
Atypical – only 2 different zones and poorly-defined
border
Target lesions favour acrofacial sites; extremities and face
Painful/pruritic
Targets may blister
+/- mucous membranes

43. Classic targets / iris – 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
Typical +/- atypical papular Typical > atypical targets
targets Severe mucosal and systemic
Little or no mucosal
features
involvement
No systemic symptoms
In all EM, majority lesions
will develop within 24 hr (all
by 72 hr)
Duration episode approx.
2/52

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

49. Erythema Multiforme - aetiology
Infection:HSV(most common ), mycoplasma
Malignancy
Drugs - sulphonamides, phenytoin,
barbiturates,penicillin, allopurinol
Immunological – LE, RhA, DM, Behcet’s
Idiopathic - 50%

50. Urticaria – ITCHY!
Erythema multiforme
 Central zone is normal skin
 Central zone of epidermal
 Lesions are transient, lasting
damage (dusky, bullous,
several hours crusted)
 New lesions appear daily  Lesions 'fixed' for at least 7
 Associated with oedema hand days
and feet (angioedema)  All lesions appear within
first 72 hours
 No oedema

51. Stevens-Johnson Syndrome and Toxic Epidermal
Necrolysis

52. SJS/TEN
• Rare , acute life-threatening mucocutaneous diseases
• Overlapping features-both T-cell-mediated
• Extensive keratinocyte cell death – separation of skin
at DEJ
• Keratinocyte death via apoptosis – 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
Genetic susceptibility HLA-B12 ↑/HLA-B*5701, HLA-DR7
Failure to detoxify reactive +HLA-DQ3 = 100% predictive TEN with abacavir
intermediate drug
metabolites
Immune response to
antigenic complex
Interaction between Fas
(CD95) and its ligand on
epidermal cells triggers
apoptosis pathways and
cell death

54. SJS and TEN
• Spectrum of severity
– SJS < 10% epidermal detachment
• ≥ 2 mucosal sites
– SJS / TEN overlap 10-30%
– 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:
– Slow acetylators
– HIV (1000x)
– Lymphoma
– SLE

55. SJS
Precipitants
Drugs 50% ≥ 14/7 (NSAIDS>sulfonamides, anticonvulsants,
penicillins and tetracyclines)
Infections 50%
 Mycoplasma, Yersinia, TB, Syphilis, Chlamydia, Strep, Typhoid,
Pneumococcus; Coccidiodomycosis, Histoplasmosis; Enterov,
Adenov, Measles, Mumps, Influenza
IBD
Vaccine

56. TEN
The more widespread, the more likely drug cause (SJS 50%
drug; TEN 90%). Other causes=infections and immunisations
Drugs causing TEN: “LOV THE SAND”
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)– 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)

58. SJS Haemorrhagic crusting
Denuded lip
Bullae
Bactrim

59. Lesions initially
erythematous, dusky or
purpuric macules-
tendency to coalesce
+/- atypical targets
48M Positive Nikolsky sign
amphetamines

60. As necrosis becomes full-thickness, dusky –red
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

62. Raw and often bleeding dermis
revealed.

63. Cephalexin
RIP 24 hours

65. Clinical
Mucous membranes
90% mucosal lesions
Erosions/erythema
General
Photophobia
Painful micturition
Fever
Severe pain
Acute renal failure
Erosions lower respiratory tract/gut-BOOP

66. SJS / TEN complications
• Hypovolaemia, metabolic abnormalities, secondary
bacterial infection
• Death
– infections (S. aureus, Pseudomonas aeruginosa)
– ARDS
– multiorgan failure/thromboembolism/GI hge
• Scarring – skin, joint contractures, cornea,
conjunctiva, lacrimal ducts, oesophageal strictures,
anal strictures, vaginal, urethral meatal stenosis (eye
complications 40%)
• Mortality 1-5% SJS/25-35% TEN (+ more in elderly)

67. SJS / TEN complications
Skin
Irregular pigmentation
Eruptive naevi
Nail dystrophy
Sicca symptoms

68. SCORTEN severity-of-illness score
Sum Mortality %
Age >40 0-1 3.2
Malignancy 2 12.1
HR > 120/min 3 35.8
Initial >10% epidermal 4 58.3
detachment
Urea > 10 mmol/L ≥5 90
Glucose > 14 mmol/L
Bicarbonate < 20 mmol/L

69. Important DDx’s for SJS/TEN
• Paraneoplastic pemphigus/pemphigus vulgaris/ bullous
pemphigoid(including drug-induced) – DIF/IDIF
• Linear IgA disease (+drug-induced)-DIF/histology
• Bullous lupus erythematosus - ANA/DIF
• Stage IV acute GVHD-evolution
• Kawasaki’s disease (children)
• Staphylococcal scalded skin ∑ (frozen section/H+E)
• Acute generalised exanthematous pustulosis-self-limiting when cease drug
• SEE FEB. 2007 JAAD CME PAPER

70. Investigations in SJS/TEN
As for erythroderma
CXR/UEC/LFT’s/Coags for systemic invlt
FBC – prognosis and evidence infection
SCORETEN – Day 1 and Day 3
Biopsy – H+E/DIF +/- frozen section
Indirect immunofluorescence – exclude PNP/PV
ANA/ENA/dsDNA (before give IVIg)
Regular cultures – skin/blood/mucous membranes
Viral swabs
IgA 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 Lₒ)

72. Management SJS/TEN
Stop causative drug and all non-life-sustaining drugs
Admit to hospital
Rapid initiation-
a) Supportive
b)Specific management – controversial and evidence
is still evolving

73. Management SJS/TEN-Supportive
Admit Burns Unit/ICU
Correct/monitor fluid and electrolyte balance
Nutrition – refer – replace calories/protein/etc
Surveillance for infection- regular swabs mouth, eyes,
skin, sputum (treat based on culture results and when
signs of sepsis – NOT PROPHYLACTICALLY)
Analgesia – Pain team review
Eye care – Consult Ophthalmology-lubricant
drops/steroid drops/chlorsig
Urology/Gynae – IDC/manual exam or dermeze tampons
Mouth care – PMMW/xylocaine viscus 2%/Diprosone OV
ung/Daktarin oral gel/white soft paraffin lips

74. Management SJS/TEN-Supportive
Physio. to prevent contractures and respiratory
Skin care – gentle handling/no tapes on skin/air
mattress/non-adherent dressings (Bactigras and
Acticoat)/biologic skin equivalents reported
Care to avoid pressure areas
Thromboprophylaxis
Medic Alert Bracelet/ADR notification/counsel relatives
Proton pump inhibitors for GIT prophylaxis

75. Management SJS/TEN-
Specific/Adjunctive
• CONTROVERSIAL! Complementary – apoptosis is
rapid + irreverisble once triggered so must be early (1st
4 days)
• Corticosteroids – deleterious effect in small studies/
possible benefit recent studies? Poor outcomes 2ₒ
inadequate doses? (pulse dexa 1.5mg/kg/day for 3/7)
• CyA - ?anti-apoptotic via ↓regulation NF-κB (3-4mg/kg/day)
• Cyclophosphamide
• Infliximab
• Plasmapheresis +/- IVIg (remove drug/metabolites/cytokines)
• IVIG high dose 2-4 g/kg

76. IVIG
IVIG – 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 –
use 1g/kg for 4/7
Survival  with every
1g/kg increment in dose
(OR = 4.2)
No prospective controlled
studies with sufficient
numbers. Doses varied in
previous studies

77. •HLA-B*1502 is strongly associated with carbamazepine-induced
TEN/SJS – reported in several independent studies
•Mostly observed in patients of South-East Asian descent
•This may also be seen in drugs with structural similarity to
CBZ in patients with HLA-B*1502
•HLA-B*5801 associated with allopurinol-induced TEN/SJS
•Screening for HLA-haplotypes ideal before using these drugs in
relevant patients

79. Eczema Herpeticum/Kaposi’s varicelliform
eruption
 Widespread cutaneous infection with a virus that normally
causes localised or mild vesicular eruptions – IN A PATIENT
WITH PRE-EXISTING SKIN DISEASE
 Majority of cases are HSV-1 in atopic eczema = eczema
herpeticum
 KVE = widespread infection with other viruses (coxsackie A16,
VZV)
 Children and young adults usually (2 nd-3rd decade)
 In eczema herpeticum, majority are primary infections, but can
occur with endogenous recurrent infection (herpes labialis)
 Risk factors
 Atopic dermatitis
 Parental /close contacts herpes labialis
 Other chronic skin disorders (pemphigus foliaceus, Darier’s, Hailey-
Hailey, MF, Sezary syndrome, ichthyosis vulgaris, CIE etc)

80. Clinical features
Incubation period 10 days
Underlying skin disease
Crops of vesicles that rapidly become pustules (new
crops 5-7 days)
Lesions begin in abnormal skin +/- generalise
Painful ‘punched out’ erosions
Secondary staph infection
Monomorphic 2-3mm haemorrhagic crusts = clue
Fever onset 2-3 d after eruption –lasts 4-5/7
+/- severe constitutional Sx
Regional lymphadenopathy
Progression to potentially fatal systemic infection
rarely

82. Eczema herpeticum/KVE-clinical
features
Localised mild infections – low-grade fever and
lymphadenopathy- usually self-limiting
Recurrences may be milder than initial
episode/sometimes comparable severity

85. Treatment
Oral (or IV antivirals)
Aciclovir/famciclovir/valaciclovir
+/- Anti-staph antibiotics
Aluminium acetate soaks
Treat underlying skin disorder
Refer to Ophthalmology if periorbital
involvement

86. Any questions?