Upadacitinib Coadministered with Methylprednisolone for Effective Treatment of SJS/TEN Overlap Syndrome: A Case Report.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) overlap syndrome, characterized by extensive epidermal necrosis, represents a life-threatening severe dermatological disorder.Therapeutic agents and regimens for this condition include high-dose glucocorticoids, intravenous immunoglobulin (IVIG), plasma exchange, immunosuppressants, and TNF-α inhibitors. A study demonstrates that JAK/STAT hyperactivation-characterized by interferon signature enrichment, STAT1 phosphorylation in keratinocytes/macrophages, and subsequent GBP1/WARS1-mediated cytotoxicity-drives epidermal detachment in TEN. JAK inhibitors (including JAK1-selective agents) suppressed this pathway in murine models and achieved rapid resolution in seven TEN patients.Recent clinical studies have demonstrated the therapeutic efficacy of JAK inhibitors in SJS/TEN management. In this context, we present the case of a 54-year-old woman who presented to the hospital with a 6-day history of rapidly worsening erythema, accompanied by a 4-day history of blistering and erosion. The patient received treatment with methylprednisolone (40 mg/day, weight: 49 kg) and upadacitinib (15 mg/day for 2 weeks). Combination therapy achieves rapid disease control by halting the progression of cutaneous necrosis while enabling accelerated glucocorticoid tapering. This case underscores that the combination therapy of upadacitinib and methylprednisolone can offer a promising approach for the SJS/TEN overlap syndrome.