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What is pyogenic sterile arthritis pyoderma gangrenosum and acne syndrome?
- • PAPA is a rare autosomal dominant episodic disorder resulting from mutation of CD2-binding protein 1 ( CD2BP1 ), also known as proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1).
- • This mutation results in hyperphosphorylation of PSTPIP1 resulting in a stronger interaction between PSTPIP1 and pyrin that leads to increased IL-1β production.
- • The first episode of arthritis typically occurs between 1 year and 10 years of age and affects one joint at a time.
- • Fever is not prominent.
- • Skin manifestations typically occur later (at puberty).
- • Treatment includes glucocorticoids and IL-1 antagonists such as anakinra.
- • Not to be confused with PFAPA