Snapshot
- A 21-year-old gentleman comes to the emergency room with a painful rash all over his body, including some lesions in his mouth. He also describes feeling feverish. On physical exam, his skin has multiple bullae that sloughs off easily with a single rub. The rash covers > 30% of his body. A careful history reveals that he was recently put on lamotrigine for his epilepsy. The lamotrigine is stopped and patient is immediately admitted to the burn unit.
Introduction
- Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – two diseases on the same spectrum
- SJS: < 10% of body surface area
- TEN: > 30% of body surface area
- SJS/TEN overlap: 10-30% of body surface area
- Severe, febrile blistering disease of skin and mucous membranes
- often caused by drugs (>>> infection)
- e.g., penicillin, sulfonamides, phenytoin, carbamazepine, lamotrigine, NSAIDs
- can be caused by infection
- e.g., mycoplasma pneumonia
- often caused by drugs (>>> infection)
- Erythema multiforme (EM) is a distinct disease from SJS/TEN according to the current consensus definition
Presentation
- Symptoms
- very painful skin (vs in EM, where pain/burning is typically very mild)
- systemic signs
- fever
- dehydration
- hypotension
- Physical exam
- initially dusky red macules or patches (not raised) that progress to tense bullae and eventual skin sloughing (vs in EM, where lesions are typically papular)
- + Nikolsky sign (rubbing of skin easily causes sloughing – splitting of epidermis from dermis)
Evaluation
- Based on clinical history and symptoms
- Skin biopsy: mainly to distinguish staphylococcal scalded skin syndrome and TEN
- Labs: normal
Differential Diagnosis
- Staphylococcal scalded skin syndrome
- Graft versus host disease
- Pemphigus vulgaris
- Erythema multiforme
Treatment
- Discontinue causative agent
- Supportive care
- wound care
- fluids, electrolytes, nutrition
- Treat underlying infection
Prognosis, Prevention, and Complications
- High mortality, especially with TEN