Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 19673875.
- Also identified by DOI 10.1111/j.1365-2133.2009.09404.x.
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Abstract
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.
Medical subject headings
- Acne Vulgaris
- Antirheumatic Agents
- Arthritis
- Interleukin 1 Receptor Antagonist Protein
- Pyoderma Gangrenosum