Erythema Multiforme, Steven Johnson Syndrome, Toxic Epidermal Necrolysis

Question 1

What is the order from least to worsening severity (in terms of body surface area) of these conditions?

Answer 1

Minor EM –> Major EM –> SJS –> TEN

Question 2

What is Erythema Multiforme? What’s it characterized by?

Answer 2

Acute immune-mediated disease characterized by smaller TARGET-like lesions/urticarial papules +/- erosions or bullae involving the palms, soles, oral, genital &/or ocular mucosa

Distribution: Symmetrical

Question 3

What’s the difference between EM Minor and EM Major?

Answer 3

EM Minor: EM with zero to mild mucous disease
EM Major: EM with EXTENSIVE mucosal involvement +/- systemic sx

Question 4

What are causes of Erythema Multiforme?

Answer 4

• Infection (HSV, Mycoplasma pneumoniae)
• Pregnancy
• Malignancy
• Drugs

Question 5

How is Erythema Multiforme treated?

Answer 5

• Typically self limiting (lesions heal in 1-2 wks w/o scarring)
• Antivirals

Question 6

What are SJS and TEN?

Answer 6

Severe mucocutaneous reactions

Question 7

What are the M/C/C of SJS & TEN?

Answer 7

• Drugs like abx, NSAIDs, Allopurinolol, Antiseizure
• Infection (Mycoplasma PNA m/c in kids)

Question 8

What are especially telling findings/signs of SJS & TEN?

Answer 8

Positive Nikolsky sign (superficial sloughing of skin by applying gentle lateral pressure)

Positive Asboe-Hansen sign

Question 9

“Skin feels like it’s on fire”

Answer 9

SJS & TEN

Question 10

What are the S&S of SJS & TEN?

Answer 10

Prodrome of fever/flu-like sx then 1-3 days later mucocutaneous lesions develop. After that, get photophobia, conjunctival burning –> dysphagia
–> malaise/mylagia/arthralgia

Skin findings:
- Ill-defined, coalescing, erythematous/dusky macules with purpuric centers
- Diffuse erythema
- TENDER to the touch
- Typically starts on face and trunk and spreads symmetrically
- Hemorrhagic crusts

Question 11

Classification of SJS, SJS/TEN Overlap, & TEN based on affected surface area:

Answer 11

SJS = <10% detachment (usu. trunk, face, neck)
SJS/TEN Overlap = 10-30% detachment
TEN = >30% detachment (all over)

Question 12

How is SJS/TEN diagnosed?

Answer 12

Biopsy

Question 13

How is SJS/TEN treated?

Answer 13

SUPPORTIVE
- D/C offending agent!
- Cyclosporine
- TNF alpha inhibitors

Question 14

With TEN, what is ALWAYS present?

Answer 14

Systemic sx! like fever, hepatitis, cytopenia, LAD