Severe drug reactions + emergency derm Flashcards
Criteria for AGEP
PHFAN
- pustules (sheets of non-folicular, sterile, superficial pustules)
- histo consistent
- fever > 38
- acute progression
- neutrophilia
Causative agents for AGEP
CCCAAAMPS (Camping with Fat MC)
- Ca Ch blockers
- Cipro
- Cephalosporins
- Antifungals
- Antimalarials
- Anticonvulsants
- Macrolides
- Penicillins
- Sulfonamides
Histology of AGEP
PuSSy DEn
- perivasc lymph/neuts
- subcorneal pustules
- spongiosis
- dermal oedema
- eosinophils
What percentage of patients with AGEP have systemic involvement?
18%
- hepatic, renal, pulmonary, BM (agranulocytosis_
What is the natural history of AGEP
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
Management of AGEP
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)
Ix in AGEP
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
Causative agents for DRESS
Be Very Very MAAADS
- BRAFi
- Vancomycin (HLA 3201)
- valtrex
- Minocycline
- Azathioprine
- Allopurinol
- Anticonvulsants
- Dapsone
- Sulfonamides
Lag time for onset of DRESS
2-12 weeks
Pathophysiology of DRESS
T cell reaction (drug specific T cell reaction) HLA subtype susceptibility --- HLA3201 (vancomycin) --- HLA5802 (allopurinol) HHV6/7 reactivation
Diagnostic criteria for DRESS
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)
Ix in DRESS
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
Management of DRESS
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
Drug causes of erythroderma
CAAAN GOAL
- Ca ch blockers
- acitretin
- anllopurinol
- anticonvulsants
- NSAIDs
- Gold
- Omeprazole
- Ab’s
- Lithium
Aetiology of erythroderma
PsO, eczema (endogenous, irritant, allergic contact), drug, CTCL, PRP, Sarcoid, lupus, pemphigus foliaceous, hailey-hailey, darier’s, lichen planus….
Causative agents for SJS/TEN
SATTAAN
- sulfonamides
- allopurinol (HLA5801)
- terbinafine
- tetracyclines
- anticonvulsants (HLA1502)
- abacavir
- NSAIDs
Latency for onset of SJS/TEN
1-3 weeks
HIstology for TEN/SJS
Full thickness epidermal necrosis
Subepidermal split
Can be minimal inflammation
- eos often not seen
Prognosis of TEN/SJS - how to assess?
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%
Clinical features TEN/SJS
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
Complications of TEN/SJS
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
Mx of TEN
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
Management of acute meningococcemia (neisseria meningitidis)
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
Signs of meningococcal meningitis
Neck stiffness / meningism (Kernig's + Brudzinski) Headache Photophobia N+V fever/chills Irritability/ confusion Seizure Skin - petechiae, purpura (often stellate), necrosis