期刊
BRITISH JOURNAL OF DERMATOLOGY
卷 161, 期 5, 页码 1199-1201出版社
WILEY-BLACKWELL PUBLISHING, INC
DOI: 10.1111/j.1365-2133.2009.09404.x
关键词
anakinra; interleukin-1 receptor antagonist; pyoderma gangrenosum; PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome
类别
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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