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Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin‐1 receptor antagonist anakinra
Author(s) -
Brenner M.,
Ruzicka T.,
Plewig G.,
Thomas P.,
Herzer P.
Publication year - 2009
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2009.09404.x
Subject(s) - pyoderma gangrenosum , anakinra , medicine , dermatology , acne , arthritis , interleukin 1 receptor antagonist , immunology , receptor antagonist , antagonist , disease , receptor
Summary 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.