The treatment of neutrophilic panniculitis can be challenging. We report a patient with a difficult-to-treat neutrophilic panniculitis who had a spectacular response to a short treatment course with the IL-1 antagonist anakinra. A 61-year-woman had a 12-year history of a serious febrile neutrophilic panniculitis and a personal history of steroid- induced pancreatitis and life-threatening methemoglobinemia under antimalarials and dapsone. When she developed a new flare, she was treated successfully with a 15-day course of subcutaneously administered injections of 100 mg anakinra. This observation is the first report attesting the efficiency of anakinra in neutrophilic panniculitis. It expands the potential indications of IL-1 inhibition in the field of the neutrophilic dermatoses, or more generally, in neutrophil-mediated systemic diseases. It underscores the potential pathogenic involvement of IL-1 in those diseases, a hallmark of autoinflammation, and supports their nosologic classification among diseases involving primarily the innate immune system.

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