AN UNUSUAL PRESENTATION OF STEVENS-JOHNSON SYNDROME (SJS) IN A PATIENT WITH TWO PRIOR SJS EPISODES

Abstract

<h3>Introduction</h3> Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are a spectrum of rare, life-threatening mucocutaneous reactions, usually occurring weeks after starting a medication. They are characterized by epidermal necrolysis and detachment, leading to complications such as dehydration, sepsis, and death. SJS occurs when skin detachment involves <10% of the body surface. <h3>Case Description</h3> A 32-year-old woman presented to the emergency department for onset of oral lesions and blistering rashes affecting her hands and feet. No ocular symptoms existed. Symptoms began hours after starting naproxen and cefixime for an axillary abscess. She noted two prior reactions with similar symptoms, but milder in severity, after taking naproxen and ibuprofen. Due to severity of her mouth ulcerations and positive Nikolsky sign, cefixime and naproxen were discontinued, and prednisone started without improvement. She was admitted for possible SJS vs. DRESS and underwent shave biopsy, which revealed epidermal necrolysis, pigment incontinence, and minimal eosinophils—more consistent with SJS. After inpatient admission, the rash had spread to her elbow. She was given antihistamines, IV fluids, and pain medication; prednisone was discontinued. She was advised to avoid NSAIDs and cephalosporin antibiotics to prevent another reaction and was discharged a few days after improvement. <h3>Discussion</h3> This case highlights several important points: (1) Prednisone is indicated in DRESS, but should be used with caution in SJS because of risk of sepsis, (2) typically SJS starts weeks into therapy, but with re-exposure it may occur in hours to days, (3) though SJS may progress rapidly, some cases continue with limited involvement.