Severe drug reactions + emergency derm Flashcards

1
Q

Criteria for AGEP

A

PHFAN

  • pustules (sheets of non-folicular, sterile, superficial pustules)
  • histo consistent
  • fever > 38
  • acute progression
  • neutrophilia
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2
Q

Causative agents for AGEP

A

CCCAAAMPS (Camping with Fat MC)

  • Ca Ch blockers
  • Cipro
  • Cephalosporins
  • Antifungals
  • Antimalarials
  • Anticonvulsants
  • Macrolides
  • Penicillins
  • Sulfonamides
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3
Q

Histology of AGEP

A

PuSSy DEn

  • perivasc lymph/neuts
  • subcorneal pustules
  • spongiosis
  • dermal oedema
  • eosinophils
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4
Q

What percentage of patients with AGEP have systemic involvement?

A

18%

- hepatic, renal, pulmonary, BM (agranulocytosis_

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5
Q

What is the natural history of AGEP

A

Onset 2-5 days
Can be prodrome of burning/itch on skin
Onset in flexures - erythema, burning
Rapid progression to sheets of superficial pustules
+/- systemic involvement
Resolves over days-2 weeks after ceasing drug w superficial desquamation

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6
Q

Management of AGEP

A
Cease the drug
Consider admission
Monitor for/exclude infection
Skin cares - condys soaks/ bleach baths
TCS for itch/ irritation in the skin
Rarely require PO prednisone (care that it isn't pustular PsO)
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7
Q

Ix in AGEP

A

confirm the diagnosis - 4mm PBx for histology
consider DIF to exclude other causes (IgA pemphigus)
Swab for m/c/s + viral swab HSV1/2 PCR
Septic Screen
FBC, ELFTs (can be systemic involvement)
ESR, CRP to help w monitoring

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8
Q

Causative agents for DRESS

A

Be Very Very MAAADS

  • BRAFi
  • Vancomycin (HLA 3201)
  • valtrex
  • Minocycline
  • Azathioprine
  • Allopurinol
  • Anticonvulsants
  • Dapsone
  • Sulfonamides
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9
Q

Lag time for onset of DRESS

A

2-12 weeks

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10
Q

Pathophysiology of DRESS

A
T cell reaction (drug specific T cell reaction)
HLA subtype susceptibility
--- HLA3201 (vancomycin)
--- HLA5802 (allopurinol)
HHV6/7 reactivation
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11
Q

Diagnostic criteria for DRESS

A

RegiSCAR Abbrev

  • AROS + LELO
  • area > 50%
  • rash c/w DRESS (maculopapular, exfoliative can be scattered pustules)
  • organ involvement (see below)
  • serology negative (ANA, Hep B/C, HIV, CMV, EBV)

ORGAN - LELO

  • lymphadenopathy (2 or more basins)
  • eosinophilia (scores from >0.7)
  • atypical lymphocytosis
  • organ - LFT > 2x ULN on 2 occasions)
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12
Q

Ix in DRESS

A
Confirm diagnosis + extent
- 4mm PBx for histo, perilesional for DIF
- FBC (eosinophilia) + film (atypical lymphocytosis)
- ESR, CRP
- ELFTs
- coags
- thyroid - TFTs
- cardiac - ECG, Echo, TnI
- pulmonary - CXR, RFTs, CT
- GIT - FOB
- pancreas - lipase
- renal - urine m/c/s, Pr:Cr, blood pressure
consider imaging - CT CAP, Abdominal US
- HHV6/7 serology
- LP if symptoms

Exc DDx

  • septic screen
  • Hep B, Hep C, HIV, EBV, CMV, syphilis
  • ANA

Later

  • consider patch test
  • monitor for late complications up to 12-18 months
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13
Q

Management of DRESS

A

Cease the drug
Admit to hospital
Monitoring, obs, fluid regulation, electrolyte regulation, systemic complications
Commence PO Prednisolone (0.5-1mg/kg/day)
— consider IV methylprednisolone (500-1000mg/day for 3 consecutive days)
— aiming to weant over 2-3 months
— steroid sparing agent - IVIg, rituximab
General skin cares
TCS for symptomatic relief

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14
Q

Drug causes of erythroderma

A

CAAAN GOAL

  • Ca ch blockers
  • acitretin
  • anllopurinol
  • anticonvulsants
  • NSAIDs
  • Gold
  • Omeprazole
  • Ab’s
  • Lithium
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15
Q

Aetiology of erythroderma

A

PsO, eczema (endogenous, irritant, allergic contact), drug, CTCL, PRP, Sarcoid, lupus, pemphigus foliaceous, hailey-hailey, darier’s, lichen planus….

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16
Q

Causative agents for SJS/TEN

A

SATTAAN

  • sulfonamides
  • allopurinol (HLA5801)
  • terbinafine
  • tetracyclines
  • anticonvulsants (HLA1502)
  • abacavir
  • NSAIDs
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17
Q

Latency for onset of SJS/TEN

A

1-3 weeks

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18
Q

HIstology for TEN/SJS

A

Full thickness epidermal necrosis
Subepidermal split
Can be minimal inflammation
- eos often not seen

19
Q

Prognosis of TEN/SJS - how to assess?

A
Scorten at day 1 + 3
CAABBBS
- cancer
- age > 40
- area > 10%
- bicarb < 20
- BUN > 10
- beats > 120
- sugar > 14
0-1 = 3% mortality
2 = 12% mortality
3 = 30%
4 = 60%
5 = 90%
20
Q

Clinical features TEN/SJS

A

Can be prodrome - fever, fatigue, malaise
Atypical targetoid lesions + erythema
Distribution more central (face/chest) - vs EM more acral
Mucosal involvement - ocular, oral, nasal, genital
Desquamation
Nikolsky positive
Can be fever
Can be URT + bowel symptoms

21
Q

Complications of TEN/SJS

A

Early

  • pain
  • psychological
  • temperature dysregulation
  • electrolyte dysregulation
  • fluid dysregulation
  • anemia
  • secondary infection + sepsis
  • ocular pain, scarring (symblepheron), visual loss
  • oral pain
  • oesophageal stricture
  • reduced intake
  • malnutrition (catabolic state + reduced intake)
  • acute renal failure
  • genital scarring / synachiae
  • urinary retention
  • alopecia
  • Beau’s lines, onycholysis, onychomadesis
  • respiratory distress syndrome
  • bronchiolitis obliterans
  • ileus, diarrhoea, abdominal pain

Late

  • ocular - scarring, ulceration, entropion/ ectropion, symblepheron, visual loss, dry eyes
  • oral - pain, dysphagia, strictures
  • oesophageal scarring
  • skin - photosensitivity, pruritus, pain, erythema, eruptive naevi, dyspigmentation
  • genital - stenosis, phimosis, synachiae, dyspareunia
  • hair - alopecia (TE)
  • nails - anonychia
  • psychological
22
Q

Mx of TEN

A

Admit to ICU/ burns
Multidisciplinary - Burns, ICU, physicians, Ophthalmology, ENT, Gynae/ urology, phsyio, nursing care, dietician, psych
CEASE THE DRUG
Cease all unnecessary drugs
Obs
Monitor - fluids, bloods, infection (swabs regularly for bacterial/ viral/ candida)
Fluid status - avoid IVC unless poor intake/ reduced output - if needed, avoid involved skin, crystalloid fluids
Nutritional - high protein diet, consider NGT if reduced oral intake
Temperature - warm room etc
Skin - chlorhex/ condys baths
WSP to eroded areas, TCS (diprosone ointment) to erythematous areas
— dressings - vazgauze, mepitel (non-adherent), mepilex, silver dressings or kept in place with tubifast
— avoid trauma to the skin - blood pressure cuffs, ecg dots etc
— do not debride
Ocular - lubricative eye drops, disruption of adhesions, swabs, further guided by ophthal
Oral - avoid hot/ spicy/ sharp foods, soft toothbrush, regular mouthwash (sodibic, sucralfate), xylocaine viscous pre-meal, +/- nilstat, WSP to lips
Genitals - consider IDC, tampons with WSP, WSP +++
Analgesia as required
DVT prophylaxis
Systemic tx - inconclusive evidence, consider CsA 2mg/kg for 7-10 days, consider IVIg 0.5-1g/kg for 3 days, TNFi
Medi-alert bracelet

23
Q

Management of acute meningococcemia (neisseria meningitidis)

A

Multidisciplinary
Medical emergency - circulatory support etc
Admission to hospital
Ceftriaxone empirically
Targeted = benzyl penicillin 60mg/kg in children
Contact trace + treat with rifampicin 2 day dose
Vaccination - B, ACWY

24
Q

Signs of meningococcal meningitis

A
Neck stiffness / meningism (Kernig's + Brudzinski)
Headache
Photophobia
N+V
fever/chills
Irritability/ confusion 
Seizure
Skin - petechiae, purpura (often stellate), necrosis
25
Q

Management of acute meningococcemia (neisseria meningitidis)

A

Multidisciplinary
Medical emergency - circulatory support etc
Admission to hospital
Ceftriaxone empirically
Targeted = benzyl penicillin 60mg/kg in children
Contact trace + treat with rifampicin 2 day dose
Vaccination - B, ACWY

26
Q

Signs of meningococcal meningitis

A
Neck stiffness / meningism (Kernig's + Brudzinski)
Headache
Photophobia
N+V
fever/chills
Irritability/ confusion 
Seizure
Skin - petechiae, purpura (often stellate), necrosis
27
Q

Management of anaphylaxis

A
Stop the causative agent
DR-ABC
Adrenaline 500mcg (10mcg/kg for paeds)
Oxygen
IV access
Crystalloid fluids
Medi-alert bracelet
Remember if surgical to address the primary issue (skin cancer etc)
Letter to all drs involved in pt's care
Notify next of kin
28
Q

Clinical features of SSSS

A

Sunburn-like erythema, painful, distressed child not wanting to move/ be picked up
Superficial desquamation that starts in the flexures
Nikolsky positive
Sparing of mucosal membranes
Perioral/ nasal crusting
Fever
Heals w/o scarring over 1-2 weeks

29
Q

Pathophysiology of SSSS

A

Staph aureus infection (localised)
Release of exfoliative toxin A or B (targets Dsg 1) –> superficial desquamation at S granulosum layer)

Adults with renal impairment can get SSSS (impaired clearance of toxin) - also malignancy, poor immunity

30
Q

Mx of SSSS

A

Admit to hospital
Monitor temp/ fluids/ electrolytes/ signs of secondary infection
Swabs, snip test
Fluids to help clear toxin
General skin cares
Analgesia
Flucloxacillin, clindamycin or vanc if MRSA

31
Q

DDx of SSSS

A
TEN/SJS
AGEP
Scalding burn, sunburn
TEN-like SLE
Kawasaki
Toxic shock syndrome
GVHD
32
Q

Toxic shock syndrome mechanism/pathophysiology

A

Staph / Strep (toxic shock-like syndrome)
TSS-toxin 1/ exotoxin C - superantigen mediated cytokine release storm w T cell activation + macrophages

Often in setting of FB (tampon, surgical mesh)

33
Q

Diagnostic criteria for TSS

A

DR FOCH +C

  • desquamative (acral) 1-2 weeks after onset
  • rash - sunburn-like diffuse macular erythema
  • fever > 39
  • organ involvement (3+) - liver, renal, GIT, haem, MSK, CNS
  • cultures negative (blood cultures, CSF, serology)
  • hypotension
  • conjunctival erythema
34
Q

Management of TSS

A
Remove the source
Admit to hospital
Haemodynamic support
IV Ab's - clindamycin targets the toxin production (Clinda cleans up the mess)
Controversial regarding oral pred/ IVIg
35
Q

Management of skin necrosis following filler injection

A
CEASE if pain/ blanching
Reassure that this often still has an ok outcome
Warm compress
Massage
Hyaluronidase infiltration
Aspirin
Topical GTN
Other vasodilators
Risk of scarring, dyspigmetnation, textural changes - can help with laser later etc
Medical indemnity
Photos
Documentation
Systems assessment - why did this occur, how can it be prevented
36
Q

Management of eye pain during periorbital filler injection

A

Stop injecting
Advise the patient - this is an emergency
if HA - flood the area with hyaluronidase - 80units/ml concentration
Warm compress + massage
Ocular massage
Oral aspirin
Topical GTN 2%
Call the ambulance - call ahead to ophthalmology
Medical indemnity
Documentation
Follow up on patient
Systems assessment - why did this occur, how can it be prevented

37
Q

Management of skin necrosis following filler injection

A
CEASE if pain/ blanching
Reassure that this often still has an ok outcome
Warm compress
Massage
Hyaluronidase infiltration
Aspirin
Topical GTN
Other vasodilators
Risk of scarring, dyspigmetnation, textural changes - can help with laser later etc
Medical indemnity
Photos
Documentation
Systems assessment - why did this occur, how can it be prevented
38
Q

Kawasaki’s criteria

A

CRASH + Burn

  • Fever >39 for 5 days, unresponsive to antipyretics
  • conjunctival erythema (non-exudative)
  • rash (non vesicular/ non bullous)
  • adenopathy (classically, single, cervical)
  • Strawberry tongue, chelitis/fissured lips
  • Hand + foot oedema/erythema/ desquamation
39
Q

Pathophysiology of Kawasaki’s

A

Unknown, possibly related to unidentified infectious agent in susceptible host (Genetics involved)
Medium vessel vasculitis

40
Q

Clinical features of Kawasaki’s

A

As per the criteria (CRASH + burn) + irritability, arthralgia, abdominal pain, malaise
4 stages
- acute (2 weeks) febrile
- 2nd phase (4-6 weeks) - risk of aneurysm
- 3rd phase convalescence (up to 3/12) - risk of MI
- chronic (variable) - risk of aneurysm later in life

41
Q

Complications of Kawasaki’s

A
Aneurysm or coronary vessels (20% reduced to 5% with treatment)
--- this is leading cause of death
MI (1-2%) 
Pericarditis
Valvular disease
42
Q

Prognostic workup for Kawasaki’s

A

We Protect Cardiac HAAM

  • WBC elevated > 12
  • PLT low <35
  • CRP elevated >3
  • HCT low < 35%
  • Albumin low < 3.5
  • Age < 12 months
  • Male gender

Reported 4 out of 7 = indication for IVIg

43
Q

Ix in Kawasaki’s

A
FBC
ELFTs
ESR, CRP
Blood cultures
Septic screen
Swabs
Serology - EBV, CMV, measles, strep serology
Cardiac - ECG, TnI, Echo, BNP, CT angiography
44
Q

Mx of Kawasaki’s

A

Admit to hospital
Mutlidisciplinary
Monitoring
IVIg 2g/kg single dose over 12H, repeat if ongoing fevers at 48H
Aspirin 30-100mg/kg until afebrile for 48H then reduce to3-5mg/kg/day for 8 weeks
Longterm monitoring
Other options - high dose prednisone 2mg/kg/day or methylpred 30mg/kg up to 1g for 3 consecutive days, infliximab, etanercept, CsA