Journal
BRITISH JOURNAL OF DERMATOLOGY
Volume 161, Issue 5, Pages 1199-1201Publisher
WILEY-BLACKWELL PUBLISHING, INC
DOI: 10.1111/j.1365-2133.2009.09404.x
Keywords
anakinra; interleukin-1 receptor antagonist; pyoderma gangrenosum; PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome
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The triad of sterile pyogenic arthritis, pyoderma gangrenosum and acne is known by the acronym of PAPA syndrome. It is a rare autosomal dominant disease of early onset. The treatment of pyoderma gangrenosum is challenging as there is often only partial response to systemic glucocorticosteroids and immunosuppressive therapies. We report the rapid and lasting response of pyoderma gangrenosum to the targeted treatment with the recombinant human interleukin-1 receptor antagonist (rHuIL-1Ra) anakinra in a patient with PAPA syndrome.
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