Delayed presentation of pembrolizumab-induced SJS-TEN overlap syndrome

Abstract

Introduction:

Immune checkpoint inhibitors (ICIs), such as pembrolizumab, are integral to cancer therapy but can cause severe immune-related adverse events (irAEs), including Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). These rare, life-threatening reactions typically occur early in treatment, with delayed onset being less commonly reported.

Case Report:

We present a 67-year-old female with triple-negative breast cancer who developed a diffuse, painful rash five months after discontinuing pembrolizumab. Physical exam revealed ∼15% body surface area involvement without mucosal lesions. Biopsy confirmed SJS/TEN overlap. The patient had previously experienced pembrolizumab-induced Grade 2 pneumonitis.

Management & Outcome:

Initial treatment with high-dose corticosteroids yielded minimal improvement. Intravenous immunoglobulin (IVIG) was added, resulting in clinical stabilization. The patient was discharged after 11 days with a steroid taper and showed gradual symptom improvement at one-month follow-up.

Discussion:

This case highlights an exceptionally delayed onset of pembrolizumab-induced SJS/TEN overlap, extending known timelines for irAE manifestation. Prior pneumonitis may have predisposed the patient to subsequent cutaneous toxicity. Despite a SCORTEN score predicting high mortality, aggressive immunosuppressive therapy led to a favorable outcome. This underscores the importance of prolonged vigilance and multidisciplinary management in patients with prior irAEs.