Case Reports in Dermatology · Published 2026-06-08 · DOI 10.1159/000552623
Lu Yang, Jiayi Ying, Xiuxiu Wang, Chengfeng Zhang, Ruiming Hu, Ning Yu
Introduction: Acute generalized exanthematous pustulosis (AGEP) is a rare but severe cutaneous adverse drug reaction characterized by the sudden onset of widespread sterile pustules on an erythematous and edematous base, often accompanied by fever and neutrophilia. Over 90% of cases are drug-induced. Its management can be particularly challenging in patients with autoimmune diseases such as systemic lupus erythematosus (SLE), where cutaneous manifestations and immune dysregulation may confound clinical assessment. Case Presentation: We report a case of severe hydroxychloroquine-induced AGEP in a patient with active SLE. The disease was refractory to systemic corticosteroids and further worsened after IVIG infusion. Given the emerging role of IL-36 pathway dysregulation in pustular dermatoses, the patient was treated with spesolimab, a monoclonal antibody targeting the IL-36 receptor, resulting in rapid defervescence and near-complete resolution of pustules within days. Conclusion: This case underscores the need for prompt recognition of drug-induced AGEP in patients receiving antimalarials or other immunomodulatory agents for connective tissue diseases. Crosstalk between IL-36 signaling and neutrophil extracellular traps (NETs) may amplify inflammation, linking AGEP with autoimmune pathology. IL-36 inhibition with spesolimab represents a potential rescue therapy for severe, treatment-refractory AGEP, particularly in patients with underlying autoimmune disorders where conventional therapies fail.
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Yang, L., Ying, J., Wang, X., et al. (2026). When Conventional Therapy Fails: Interleukin-36 Inhibition for Severe Hydroxychloroquine-Induced Acute Generalized Exanthematous Pustulosis in Systemic Lupus Erythematosus – A Case Report. Case Reports in Dermatology. https://doi.org/10.1159/000552623