Anakinra — Perhaps, a New Therapy for Pyoderma Gangrenosum?

Pyoderma gangrenosum (PG) is often difficult to treat. Occasionally, PG appears with arthritis and acne in a genetic disorder known as PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne syndrome). PAPA is an autosomal dominant disorder caused by mutations in the proline-serine-threonine phosphatase interacting protein 1 gene (PSTPIP1). The disorder is a member of a group of hereditary autoinflammatory diseases that includes familial Mediterranean fever, cryopyrin-associated periodic syndromes, and Muckle-Wells syndrome. PSTPIP1 mutations lead to elevated levels of interleukin-1β. Anakinra is a recombinant human IL-1 receptor antagonist, and its use lowers the level of IL-1β; these authors report the successful use of anakinra in a pat…