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Zhenwu Lin, John P. Hegarty, Tony Lin, Barbara Ostrov, Yunhua Wang, Wei Yu, Ashley A. Kelly, Lisa S. Poritz, Walter A. Koltun, Failure of anakinra treatment of pyoderma gangrenosum in an IBD patient and relevance to the PSTPIP1 gene, Inflammatory Bowel Diseases, Volume 17, Issue 6, 1 June 2011, Pages E41–E42, https://doi.org/10.1002/ibd.21684
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To the Editor:
Pyoderma gangrenosum (PG) is a skin disorder affecting approximately 2%-5% of patients with inflammatory bowel disease (IBD). PG can also be part of PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, and acne),1,2 which is an autosomal dominant disease caused by mutations A230T and E250Q in the threonine phosphatase-interacting protein 1 (PSTPIP1; CD2BP1) gene on chromosome 15.3 The PSTPIP1 mutations lead to elevated levels of interleukin-1β and have been successfully treated with the recombinant human interleukin-1 receptor antagonist, anakinra.1 Recently a microsatellite variation in the promoter region of PSTPIP1 has been also associated with Crohn's disease (CD) and aseptic abscesses.4 We investigated the case of a 42-year-old male diagnosed with ulcerative colitis at the age of 31 who underwent ileal pouch anal anastomosis for medical failure (including failing cyclosporine and infliximab) with the postoperative complication of severe peristomal PG that resolved with stoma closure but then recrudesced 3 years later when he was again given a stoma for proximal small bowel to pouch fistulizing disease. His diagnosis was changed to CD but his severe, near circumferential peristomal PG was nonresponsive to steroid and infliximab treatment. We speculated that this patient may harbor a PSTPIP1 mutation and therefore be responsive to anakinra treatment.
