Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra.
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.
Brenner
M
M
Department of Dermatology, Ludwig-Maximilian-University of Munich, D-80337 Munich, Germany. Michaela.brenner@med.uni-muenchen.de
Ruzicka
T
T
Plewig
G
G
Thomas
P
P
Herzer
P
P
D000152:Acne Vulgaris / Q000188:drug therapy*; D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001168:Arthritis / Q000188:drug therapy*; D006801:Humans; D007279:Injections, Subcutaneous; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D017511:Pyoderma Gangrenosum / Q000188:drug therapy*; D013577:Syndrome; D016896:Treatment Outcome
D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein