44 - Epidermal Necrolysis (SJS and TEN)

Question 1

Highest incidence of EN in

Answer 1

Adults after 65 years of age

Question 2

Y/N: Men and women are equally affected by EN.

Answer 2

N - women more frequently affected

Question 3

Prognosis score for EN

Answer 3

SCORTEN

Question 4

Most important etiologic factor in EN

Answer 4

Drugs

Question 5

High-risk drugs in EN

Answer 5

Antibacterial sulfonamides
Aromatic antiepileptic drugs
Allopurinol
Oxicam NSAIDs
Lamotrigibe
Nevirapine

Question 6

Risk of EN seems to be confined to the first _____ weeks of treatment

Answer 6

8

Question 7

Extreme phenotype of LE that has to be considered as a differential diagnosis of EN

Answer 7

Erosive LE

Question 8

Cytotoxic T cells kill keratinocytes directly and indirectly through the recruitment of other cells that release soluble death mediators, the principal being

Answer 8

Granulysin

Probably also IL-15

Question 9

Medications taken to treat the prodromal symptoms of EM are often accused to have caused the reaction

Answer 9

Protopathic bias

Question 10

Dislodgement of the epidermis by lateral pressure on erythematous zones

Answer 10

Nikolsky sign

Question 11

Represents a little less than 1% of the BSA

Answer 11

One hand (palms and fingers)

Question 12

Low serum bicarbonate levels usually result from _____ and more rarely from _____

Answer 12

Respiratory alkalosis related to the specific involvement of bronchi
Metabolic acidosis

Question 13

Often considered to be an unfavorable prognostic factor but is too rare to have a significant impact on SCORTEN

Answer 13

Neutropenia

Question 14

Biopsy should preferentially not be taken from

Answer 14

Legs below the knee

Could be superimposed by stasis dermatitis

Question 15

Absence of _____ involvement or its restriction to a single site must always raise the suspicion of an alternative diagnosis to EN

Answer 15

Mucous membrane

Question 16

Hallmarks of generalized bullous fixed drug eruption

Answer 16

Prior events
Rapid onset after drug intake
Very large, well-demarcated blisters

Question 17

During the acute phase, the most common complication of EN is

Answer 17

Sepsis

Question 18

Main causes of mortality in EN

Answer 18

Infections

Question 19

Most frequent pathogens of infections in EN

Answer 19

S. aureus

Pseudomonas spp

Question 20

Most frequent sequelae of the skin in EN

Answer 20

Hypo- and hyperpigmentation

Question 21

The epidermal detachment progresses for

Answer 21

5-7 days

Question 22

The plateau phase corresponds to

Answer 22

Progressive reepithelialization

Question 23

In case of doubt, all not life-sustaining drugs should be stopped when initiated and administered within the previous

Answer 23

8 weeks

Question 24

Y/N: Volumes of infusions are usually more than for burns of similar extent of skin detachment.

Answer 24

No - interstitial edema is absent in EN vs burns

Question 25

Environmental temperature should be raised to

Answer 25

28-30 deg C

Question 26

Prophylactic _____ are not indicated

Answer 26

Antibiotics

Question 27

Prophylactic _____ should be provided during hospitalization

Answer 27

Anticoagulation

Question 28

Only feasible in populations where a strong association has been established between a simple genetic marker and the risk of EN

Answer 28

Primary prevention

Question 29

HLA-_____ in EN induced by carbamazepine in persons of Southeast Asian ancestry

Answer 29

B*1502

Question 30

Important for patients who experienced EN and are reluctant to take any medication

Answer 30

Secondary prevention