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(Ch20) EM and SJS/TEN Flashcards

1
Q

Rashes that increase in Spring and fall

A

EM & PR

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2
Q

Most common cause of EM?

A

infections 90%
MC HSV1>HS2
followed by mycoplasma pneumonia

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3
Q

what percentage of EM preceded by herpes labials?

A

50%

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4
Q

other name of EM minor caused by HSV?

A

Von Hebra’s

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5
Q

most common cause of EM major

A

Mycoplasma pneumonia

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6
Q

Mycoplasma pneumonia EM clinical features?

A

Severe Mucocutanous involvement with atypical papular targets

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7
Q

genetic associations with EM

A

HLA-DQw3
HLA-DRw53
HLA-Aw33

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8
Q

causes/triggers of EM?

A
  1. Infections 90%
    viruses:
    - HSV 1,2
    - Parapox virus(orf)
    -VZV, EBV

Bacteria:
-Mycoplasma pneumonia
-Chylamydia
- TB

fungal:
-Histoplasma capsulatum

  1. Drugs <10%
  2. physical triggers (trauma, UV and Clod ) kobener must be prior rash not after
  3. poison Ivy and vaccines
    5.lupus & Bechet
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9
Q

which trigger or infection As/w Erythema nodosum

A

Histoplasma capsulatum

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10
Q

Drugs that can trigger EM?

A

SPAAN
Sulfa
Penicillin
Allopurinol
Anticonvulsants
NSAIDs

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11
Q

Pathogenesis of mycoplasma EM

A

molecular mimicry

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12
Q

which cell mediate HSV triggered EM

A

virus DNA transmitted by CD34+ to Th1 which releases IFN gamma

patients have normal immunity but may have difficulty clearing virus infected cells

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13
Q

Classic Primary lesion of EM

A

Typical targets
(3 zones)
1. center: dusky , vesicular or necrotic
2. pale oedematous rim
3. erythematous macule

Favours Face and distal extremities

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14
Q

Clinical lesions or features of EM

A

Typical targets
Atypical Papular targets (2 Zones)
+/- Mucosal involvement

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15
Q

what type of atypical targets present in EM vs SJS/TEN

A

Atypical Papular targets –> EM

Atypical Macular Targets–> SJS/TEN

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16
Q

Most common Location for EM rash

A

Dorsal hands and forearms

(UL>LL)
(distal > proximal)
face is a common location as well

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17
Q

Can EM progress to SJS?

A

No

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18
Q

clinical criteria to differentiate EM vs SJS/TEN

A

1.Type of lesions (typical and papular atypical targets)

  1. (Topography) Acrofacial distribution in EM

3.Abscence/presence of overt Mucosal involvement

  1. Path: ↓Eosin, ↓ epidermal necrosis
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19
Q

what are the systemic sx of EM

A

Fever and Arthralgia

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20
Q

EM Minor vs Major

A

both have targets (typical and papular atypical) and same distribution

Minor: no or mild mucosal involvement and no systemic sx

Major: Severe mucosal + systemic sx

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21
Q

oral variant of EM

A

MC in mid aged F
limited to oral cavity

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22
Q

MC mucosal location of EM

A

lips and buccal mucosa >ocular and genital

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23
Q

Earliest path sign of EM

A

Apoptosis of keratinocytes

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24
Q

Path of EM

A

basal Vacuolar interfacce dermatitis with apoptosis, spongosis and dermal inflammation and

absent or rare Eosin

normal SC. +/- vesicle

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25
Path EM vs SJS/EM
EM: ↓Eosin, ↓ epidermal necrosis, ↑↑dermal inflammation
26
percentage of EM patient with HSV +ve PCR?
80%
27
Which part of the lesion to swab for HSV?
early papule or outer rim
28
how long does it take for EM to completely developed
72h
29
prognosis of EM?
Spontaneously resolves within 2 weeks without sequela Severe mucosal may persist up to 6 weeks ocular complications if not treated eye
30
Clinical course of EM?
abrupt onset 24h fully developed 72h resolves 2 weeks
31
Time limit to use antiviral ?
prior to sx after sx ineffective
32
prophylactic Rx for EM?
Valtrex 1g/d for at least 6 months
33
Rx for EM
Mild Sx tx Severe SCS/CsA/MMF/AZA consider oral antihistamine for pruritus antiseptic cream for rash wound care eye care oral rinses/anethetics /antiseptics
34
EM vs Urticaria
EM: 1 -lesion's centre damaged skin 2 -Fixed lesions 3 -fully developed 72h 4- no angiodema Urticaria: 1 -centre normal skin 2 - transient lesions 3 - daily new lesions 4- +/-Angiodema
35
HSV recurrent EM frequency ?
2/year
36
RIME clinical features?
Clinical RIME: 1- Mucosal: Severe mucositis ≥2 mucosal sites 2-cutanous: -Vesiculobullous or atypical targets - +/- sub corneal pustules - <10% BSA
37
RIME prognosis ?
<5% ICU 10% ocular complications
38
labs for RIME?
Mycoplasma: - IgM: repeat multiple times -IgG: 4 folds increase
39
Ddx for EM
Urticaria Serum sickness/like reaction Fixed drug eruption
40
Target lesion ddx
FREE Vanilla Sweet at target: -Fixed Drug eruption -Rowell syndrome /SLE - EAC &EM - Eruption light -Vasculitis/Kawasaki - Sweet syndrome & Sjs
41
epidemiology of SJS/TEN
MC F elderly SJS 1-6/mil TEN 1/mil annul incidence
42
Risk factors of SJS/TEN
1. Slow Acetylators 2. Immunodeficent (AIDS,Lymphoma) 3. combo RadioTx+ Aromatic Anticonvulsants 4. HLA genes
43
Which HLAs As/w Carbamazepine
HLA-B1502- Asians HLA-B 3101- Europeans
44
HLA associated with Abacavir
HLA-B 5701
45
HLA As/w Allopurinol in Han Chinese
HLAB- 5801 han Chinese
46
Which HLA As/w ocular complications in white?
HLA-B-DQ1
47
Main mediator of Apoptosis in SJS/TEN
Granulysin
48
Factors involved in pathogenesis of SJS/TEN
1. Drugs 2. Granulysin 3. FasL (induce caspases) 4. Granzyme B and Perforins (holes and induce caspases) 5. Annexin 1
49
Drugs inducing SJS/TEN
1-3 weeks (earlier with re-exposure) SPAAAN Slufonamides Penecillin Anticonvulsant **Allopurinol** Anti-retovirals NSAIDs Sulfonimides abx don't cross react with non abx sulfonmides (HCTZ)
50
causes of SJS/TEN
almost always drugs but can be caused infections (same EM) and contrast The Longer half life of the drug the higher risk of SJS/TEN
51
time interval for SJS/TEN after drug
1-2 weeks
52
Which meds can have delayed SJS/TEN up to 2 months after initiation?
Anticonvulsants
53
Which anticonvulsant that does not cross react with aromatic anticonvulsant
Lamotrigine
54
Which anticonvulsant that does not cross react with other anticonvulsant
Valoproic Acid
55
SJS vs TEN
BSA SJS<10% Overlap 10-30% TEN>30%
55
Which meds has priority to DC other than culprit drug ?
Long hlaf life meds
56
How to calculate BSA in SJS/TEN
Calculate detachable skin only (+ve Nikolsky)
57
What type of targets in SJS/TEN
Atypical **Macular** targets
58
What part of the body spared in SJS/TEN?
Distal extremities (except palms & soles)
59
what type of blisters in SJS/TEN?
Flaccid that detach easily
60
Clinical Signs of detachable skin?
Niklosky: detachable with Tangential pressure Asboe-Hansen: detachable with vertical pressure
61
What percentage of Respiratory involvement?
25%
62
Relative contraindications for IVIg?
Hypercoagulable state IgA deficiency Renal disease
63
Three different concepts of neoantigenic drug–tissue complex is formation?
1. Hapten/pro-hapten concept (covalent) 2. p-i concept (non covalent) 3. Altered peptide concept
64
Earliest path sign of SJS/TEN
Apoptosis of individual keratinocytes
65
Early and late path signs of SJS/TEN
Early: Apoptosis of individual keratinocytes Late: full epidermal necrosis both has eosin and sparse dermal lymph
66
Scores to evaluate SJS/TEN severity ?
1. SCORTEN (superior) 2. ABCD-10 (has x3 points for dialysis)
67
SCORTEN
TAMEBUG each 1 point - Tachycardia >120 - Age >40 - Malignancy - Epidermal loss >10% - Bicarbonate <20 -Urea >27 - Glucose >250
68
Mortality rate for scores
0-1-> 3% 2 -> 12% 3 ->35% 4 -> 58% 5 -> >90%
69
Most important single risk factor for mortality ?
Bicarbonate <20
70
Which factor correlate with prognosis ?
rapidity of drug DC
71
impact of early drug DC?
30%/day reduction. in mortality
72
Systemic meds for SJS/TEN?
- high dose IVIG (block fas receptor) - TNFi Etanerecept - Cyclosporine - Dexamethasone
73
Most common complication ?
Ocular (80%) -Dry Eye syndrome (MC) -Entropion -Blindness - persistent erosions
74
other Complications of SJS/TEN
Ocular Resp Genital Psych electrolytes imbalance insulin abnormality hair loss nail loss eruptive nevi Death
75
Most common cause of Death?
infection (Staph and Pseudomonas)
76
Prognosis of SJS/TEN
Mortality rate - SJS 5% - TEN 30%
77
When to measure SCORTEN
Day 1 and 3 of hospitalization
78
Serologic test to differentiate SJS/TEN from morbiliform eruption
Granulysin and HMGB1
79
Benzodiazepine As/w SJS/TEN
Clobazam
80
Infection that increase risk of SJS?
HIV
81
type of bed sheet in SJS/TEN
Aluminium bed sheet
82
Why no need to graft the skin for SJS/TEN?
Complete Re-epitheliazation within 3 weeks
83
Ddx for SJS/TEN
EM gen bollous drug eruption linear IgA bollous TEC SSSS DRESS
84
Wound care for SJS
Vaseline gauze/silicon non adherent dressing over detached skin and pressure areas face with NS and mupirocin nostrils incase of maceration use silver nitrate
85
frist location for SJS/TEN rash
Trunk mostly and then face and other body
86
Mention five infectious ethologies other than viral that can precipitate Erythema Multiforme?
Mycobacterial infection Mycoplasma pneumoniae Histoplasmosis Dermatophytes Salmonella
87
Mention five contributing factors to increase mortality in Toxic Epidermal Necrolysis?
1. Infection and sepsis 2. Transepidermal fluid loss and electrolytes imbalance 3. Insulin resistance and decrease insulin secretion 4. Hypercatabolic state 5. Multi organ failure and Acute respiratory distress syndrome
88
What is the most important step in managing Toxic Epidermal Necrolysis?
Removal of the culprit drug is mainstay of treatment.
89
What cytokines correlates with severity and mortality in Toxic Epidermal Necrolysis?
IL-15 level correlate with severity and mortality in Toxic Epidermal Necrolysis.
90
19- What is the earliest Clinical sign of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis?
Fever, stinging eyes, and painful swallowing are the earliest sign of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis. They precede cutaneous manifestations by 1-3 days

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