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Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Update In Therapeutic Approach Dr Oh Choon Chiat Associate Consultant Department of Dermatology Singapore General Hospital

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Page 1: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis:

Update In Therapeutic Approach

Dr Oh Choon Chiat Associate Consultant

Department of Dermatology Singapore General Hospital

Page 2: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 3: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

Adverse drug reactions

• Exanthematous

• Fixed drug eruption

• Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related eosinophilia with systemic symptoms (DRESS)

• Epidermal necrolysis: Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN)

Page 4: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 5: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

What type of drug reaction is this?

a. Drug-Induced Hypersensitivity Reaction b. Exanthematous c. Fixed Drug Eruption d. Stevens-Johnson Syndrome e. Vasculitis

Page 6: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 7: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

Exanthematous eruptions

• Most common of all cutaneous drug eruptions (~90%) • Limited to the skin • Lesions initially appear on the trunk and spread centrifugally to the

extremities in a symmetric fashion • Erythematous macules and infiltrated papules • Pruritus and mild fever may be present • Skin lesions usually appear more than 2 days after the drug has been

started, mainly around day 8-11, and occasionally persists several days after having stopped the drug

Page 8: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 9: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

What’s the likely diagnosis?

1. Bullous pemphigoid

2. Erythema migrans

3. Fixed drug eruption

4. Spider bite

5. Vasculitis

Page 10: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 11: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

Fixed Drug Eruption

• Adverse drug reaction characterized by the formation of a solitary erythematous patch or plaque that will recur at the same site with re-exposure to the drug

• This distinguishing feature is why it’s called “fixed”

• Commonly involved drugs include: phenolphthalein (laxatives) /barbiturates / tetracyclines/ metronidazole/ sulfonamides/ NSAIDs

Page 12: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 13: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 14: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 15: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

What is the next best step in management? 1. Consult dermatology

2. Discontinue all non-life-sustaining medications

3. Request a tissue biopsy to confirm suspected diagnosis

4. Consider transfer to burn unit

5. All of the above

Page 16: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

HOW TO BREAK BAD NEWS TO A DERMATOLOGIST

Page 17: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 18: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 19: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 20: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 21: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 22: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 23: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 24: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 25: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 26: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 27: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 28: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 29: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 30: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 31: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 32: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 33: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 34: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 35: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 36: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 37: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 38: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 39: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 40: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 41: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 42: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

Summary

• A detailed medication history is essential in suspected drug reactions

• Document the drug reaction in the patient’s chart with the medication and description of the reaction

• Exanthematous eruptions are the most common of all cutaneous drug eruptions and tend to resolve without sequelae

• Fixed drug eruptions will recur at the same with re- exposure to the drug

Page 43: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related

Summary

• SJS and TEN are acute life-threatening mucocutaneous reactions characterized by extensive necrosis and detachment of the epidermis and mucosal surfaces

• Consult dermatology at the earliest moment of concern for SJS/TEN

• Cessation of culprit drug/ supportive care of skin and affected organs/ IVIG vs Ciclosporin

Page 44: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 45: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 46: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related
Page 47: AMS - Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Adverse drug reactions • Exanthematous • Fixed drug eruption • Drug-induced hypersensitivity syndrome (DIHS)/ Drug-related