Patient Case: Stevens-Johnson Syndrome (SJS)
A 40-year-old man presents to the emergency department with a painful rash, fever, and mucosal involvement. His symptoms started five days ago with flu-like symptoms, including fever, fatigue, and sore throat. Two days later, he developed a red, blistering rash that rapidly spread across his face, trunk, and extremities. He also has painful mouth ulcers, red eyes, and difficulty swallowing.
His medical history is significant for hypertension, for which he recently started carbamazepine for trigeminal neuralgia two weeks ago. He denies any recent infections, travel, or new food allergies.
On examination, he appears ill and in distress. He has dusky red macules with blistering and peeling skin affecting about 10% of his body surface area (BSA). There is mucosal erosion of the lips and oral cavity, with conjunctival redness. A positive Nikolsky sign is noted.
A diagnosis of Stevens-Johnson Syndrome (SJS) secondary to carbamazepine use is made. The patient is immediately admitted to the intensive care unit, the offending drug is discontinued, and supportive treatment, including fluid replacement, pain management, and ophthalmologic evaluation, is initiated.
- What is the most likely diagnosis for this patient’s condition?
a) Toxic epidermal necrolysis (TEN)
b) Stevens-Johnson Syndrome (SJS)
c) Erythema multiforme minor
d) Staphylococcal scalded skin syndrome - What is the most likely trigger for this patient’s condition?
a) Recent bacterial infection
b) Carbamazepine use
c) Autoimmune disease
d) Food allergy - Which of the following clinical findings is most characteristic of Stevens-Johnson Syndrome?
a) Thick, scaly plaques on extensor surfaces
b) Superficial bullae with honey-colored crusts
c) Dusky macules with blistering, mucosal involvement, and a positive Nikolsky sign
d) Itchy, annular lesions with central clearing - What is the most appropriate immediate management for this patient?
a) Systemic corticosteroids and antibiotics
b) Discontinuation of the offending drug and supportive care
c) Skin biopsy and antifungal therapy
d) Surgical debridement of necrotic skin
Answers
- (b) Stevens-Johnson Syndrome (SJS)
- SJS is characterized by fever, flu-like symptoms, and a painful blistering rash that affects <10% of the body surface area (BSA). The presence of mucosal involvement and a positive Nikolsky sign supports this diagnosis. If >30% BSA is affected, the condition is classified as toxic epidermal necrolysis (TEN).
- (b) Carbamazepine use
- Drugs, especially anticonvulsants (e.g., carbamazepine, lamotrigine), sulfonamides, and NSAIDs, are the most common triggers of SJS. This patient recently started carbamazepine, making it the likely cause.
- (c) Dusky macules with blistering, mucosal involvement, and a positive Nikolsky sign
- SJS presents with targetoid macules that progress to epidermal detachment, oral ulcers, and conjunctivitis. The Nikolsky sign (skin detachment with slight pressure) is a key diagnostic feature.
- (b) Discontinuation of the offending drug and supportive care
- Immediate withdrawal of the causative drug is critical. Patients require intensive supportive care, IV fluids, pain control, ophthalmologic evaluation, and wound care. Corticosteroids are controversial and generally avoided.
