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Title: What's the diagnosis? Written by Dr. Ari Kestler

Category: Visual Diagnosis

Posted: 6/27/2011 by Haney Mallemat, MD

49 y.o. female on Trimethoprim/sulfamethoxazole presents with rash and oral mucus membrane lesions. Diagnosis?

Answer: Steven-Johnson Syndrome

Malaise and fever prodrome, followed by erythematous or purpuric macules and plaques
- <10% body-surface area (BSA) and mucosal membrane involvement (e.g., ocular, oral, genital)
- Toxic Epidermal Necrolysis defined as >30% BSA involvement
Immune-complex hypersensitivity reaction from viral, bacterial, chemical, or drug exposure.
- Common medications:
  - Antibiotics; Sulfonamides > PCN > Cephalosporins
  - NSAIDs
  - Anti-psychotics
Management:
- Remove possible trigger(s)
- Treat like burns; supportive care (hemodynamic support, electrolyte management, analgesia)
- Strongly consider Burn ICU.

French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int. Mar 2006;55(1):9-16

Schöpf E. Toxic epidermal necrolysis and Stevens-Johnson syndrome. An epidemiologic study from West Germany. Arch Dermatol. 1991;127(6):839.

Roujeau JC. Severe adverse cutaneous reactions to drugs. N Engl J Med. 1994;331(19):1272.

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