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.

1.
Sutra-Loubet C, Carlotti A, Guillemette J, Wallach D: Neutrophilic panniculitis. J Am Acad Dermatol 2004;50:280–285.
2.
Lachmann HJ, Kone-Paut I, Kuemmerle- Deschner JB, et al: Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med 2009;360:2416–2425.
3.
Kieffer C, Cribier B, Lipsker D: Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 cases and review of the literature. Medicine 2009;88:23–31.
4.
Frossard JL, Steer ML, Pastor CM: Acute pancreatitis. Lancet 2008;371:143–152.
5.
Ash-Bernal R, Wise R, Wright S: Acquired methemogloblinemia. A retrospective series of 138 cases at two teaching hospitals. Medicine 2004;83:265–273.
6.
Oh JS, Kim SU, Oh YM, et al: The usefulness of the semiquantitative procalcitonin test kit as a guideline for starting antibiotic administration. Am J Emerg Med 2009;27:859–863.
7.
Sfia M, Boeckler P, Lipsker D: High procalcitonin levels in patients with severe drug reactions. Arch Dermatol 2007;143:1591.
8.
Church LD, Savic S, McDermott MF: Long-term management of patients with cryopyrin-associated periodic syndromes: focus on rilonacept (IL-1 Trap). Biologics 2008;2:733–742.
9.
Lachmann HJ, Hawkins PN: Developments in the scientific and clinical understanding of autoinflammatory disorders. Arthritis Res Ther 2009;11:212.
10.
Aksentijevich I, Masters ML, Ferguson PJ, et al: An autoinflammatory disease with deficiency of the IL-1-receptor antagonist. N Engl J Med 2009;360:2426–2437.
11.
Delluc A, Limal N, Puéchal X, Francès C, Piette JC, Cacoub P: Efficacy of anakinra, an IL-1 receptor antagonist, in refractory Sweet syndrome. Ann Rheum Dis 2008;67:278–279.
12.
Vounotrypidis P, Sakellariou GT, Zisopoulos D, Berberidis C: Refractory relapsing polychondritis: rapid and sustained response in the treatment with an IL-1 receptor antagonist (anakinra). Rheumatology (Oxford) 2006;45:491–492.
13.
Ghiringhelli F, Apetoh L, Tesniere A, et al: Activation of the NLPR3 inflammasome in dendritic cells induces IL-1β-dependent adaptive immunity against tumors. Nat Med 2009;15:1170–1178.
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