Guest: Stevens Johnson Syndrome, A dermatologic nightmare. Topics discussed: Severe mucotaneous reactions, distinguishing between Stevens Johnson Syndrome and Toxic Epidermal Necrolysis, prevalence and demographics of Stevens Johnson Syndrome, personal experiences with the condition and its triggers, causes and complications of Stevens Johnson Syndrome.
Stevens-Johnson syndrome and toxic epidermal necrolysis are severe mucocutaneous reactions triggered by medications, characterized by extensive necrosis and detachment of the epidermis and mucous membranes.
Promptly stopping the offending agent is crucial in management, and treatment involves immediate discontinuation of the causative medication and supportive care.
Deep dives
Symptoms and Presentation of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
The podcast episode discusses the case of a 49-year-old female presenting with a rash, fever, ulcers, and red eyes. The most common initial symptom in this condition is fever. Stevens-Johnson syndrome and toxic epidermal necrolysis are severe mucocutaneous reactions triggered by medications. They are characterized by extensive necrosis and detachment of the epidermis and mucous membranes. The presentation includes flu-like prodrome, fever, skin lesions, and mucosal involvement such as oral ulcers and conjunctivitis. The cutaneous lesions are ill-defined, erythematous macules with purpura that can be very painful and tender to touch. The palms and soles are typically not involved. The patient may exhibit a positive Nikolski sign. It is important to recognize the clinical features and make a prompt clinical diagnosis to avoid missing this serious and potentially life-threatening condition.
Causative Drugs and Mortality Rate
Causative drugs for Stevens-Johnson syndrome and toxic epidermal necrolysis include lamotrigine, phenobarbital, carbamazepine, sulfonamides, and NSAIDs. Penicillin is not a common offender. The mortality rate of these conditions is high, with approximately 38% of patients experiencing severe complications leading to sepsis, multi-organ failure, DIC, and ARDS. Thrombocytopenia can be observed in up to 10% of patients. However, laboratory tests are not helpful in establishing the diagnosis, which relies primarily on clinical evaluation. Promptly stopping the offending agent is crucial in management.
Treatment and Care
Treatment involves immediate discontinuation of the causative medication and supportive care. Patients should be transferred to a regional burn center or an ICU equipped with burn management capabilities. The Lund Browder chart can be used to assess the degree of burns. Wound care, fluid and electrolyte management, pain control, and infection prevention are essential components of therapy. Consultations with ophthalmology and gynecology may be required for eye and genital mucosal involvement. Although there is no specific curative therapy, steroids, IVIG, and immunomodulators are not recommended. Recovery time can vary, with skin sloughing typically ceasing after a week and re-epithelialization taking up to three weeks if the patient survives.
Stevens Johnson. You must be a pretty bad dude to be named after this condition...Let's talk about this dermatologic nightmare that might be the only way to get a dermatologist to come to the hospital after hours.
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Cite this podcast as: Briggs, Blake; Wosiski-Kuhn, Marlena. 195. Stevens Johnson Syndrome. October 16th, 2023. Accessed [date].
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