Pyogenic sterile arthritis pyoderma gangrenosum and acne syndrome (PAPA syndrome)

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 
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