JAK inhibition therapy for intractable cutaneous infectious granulomatosis with unknown etiology: a case report.
Suiting Ao, Mingjia Hu, Fanli Xu et al.
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Case in brief
A 36-year-old woman presented with chronic, treatment-resistant plaques on her face and legs. Histopathology supported infectious granulomatosis, but no specific pathogen was identified, including after antifungal therapy, which had not prevented worsening. Immunohistochemical analysis showed elevated cytokine levels. She received upadacitinib 15 mg daily and experienced rapid improvement followed by sustained remission, with no recurrence at the 8-month follow-up.
What made this case unusual
The granulomatous lesions were considered infectious despite the absence of an identifiable pathogen; elevated cytokine levels supported an immune-mediated component, and symptoms worsened despite antifungal therapy.
Diagnostic / clinical pearl
When granulomatous skin disease remains pathogen-negative and refractory to antifungal therapy, the findings in this case suggest considering an immune-mediated component; cytokine analysis may help characterize it.
Why it matters
This case documents sustained remission with upadacitinib in pathogen-negative cutaneous granulomatosis, suggesting a potential role for JAK inhibition in a refractory presentation.