Drug Eruptions: Bullous

Question 1

SJS & TEN

meaning

Answer 1

STEVEN-JOHNSON SYNDROME (SJS)/TOXIC EPIDERMAL NECROLYSIS (TEN)

Question 2

SJS & TEN morphology:

Answer 2

prodromal rash (morbilliform/targetoid lesions ± purpura, or diffuse erythema),
confluence of flaccid blisters, positive Nikolsky sign (epidermal detachment with shear stress), full
thickness epidermal loss; dusky tender skin, bullae, desquamation/skin sloughing, atypical targets

Question 3

SJS & TEN classication:

Answer 3

BSA with epidermal detachment: <10% in SJS, 10-30% in SJS/TEN overlap, and >30% in
TEN

Question 4

SJS & TEN spread:

Answer 4

face and extremities; may generalize; scalp, palms, soles relatively spared; erosion of mucous
membranes (lips, oral mucosa, conjunctiva, GU mucosa)

Question 5

SJS & TEN systemic features:

Answer 5

fever (higher in TEN), cytopenias, renal tubular necrosis/AKI, tracheal erosion,
infection, contractures, corneal scarring, phimosis, vaginal synechiae

Question 6

SJS & TEN time course:

Answer 6

appears 1-3 wk after drug initiation; progression <4 d; epidermal regrowth in 3 wk

Question 7

SJS & TEN

constitutional symptoms:

Answer 7

can have constitutional symptoms: malaise, fever, hypotension, tachycardia

Question 8

SJS & TEN Epidemiology

Answer 8

SJS: 1.2-6/million; TEN: 0.4-1.2/million

Question 9

SJS & TEN risk factors

Answer 9

SLE, HIV/AIDS, HLA-B1502 (reaction most prevalent in East Asians, associated with
carbamazepine), HLA-B5801 (reaction most prevalent in Asians and Caucasians, associated with
allopurinol)

Question 10

SJS & TEN common causative agents:

Answer 10

drugs (allopurinol, anti-epileptics, sulfonamides, NSAIDs, cephalosporins)
responsible in 50% of SJS and 80% of TEN; viral or mycoplasma infections

Question 11

SJS & TEN prognosis:

Answer 11

5% mortality in SJS, 30% in TEN due to fluid loss and infection

Question 12

SJS & TEN Differential Diagnosis

Answer 12

scarlet fever, phototoxic eruption, GVHD, SSSS, exfoliative dermatitis, AGEP, paraneoplastic pemphigus

Question 13

SJS & TEN Management

Answer 13

discontinue offending drug
• admit to intermediate/intensive care/burn unit
• supportive care: IV uids, electrolyte replacement, nutritional support, pain control, wound care, sterile
handling, monitor for and treat infection
• IVIg or cyclosporine or etanercept