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PAPA

PAPA syndrome. Pyogenic Arthritis, Pyoderma with AcneMoreira A, 2017. CC BY NC 4.0

Pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA)

Definition

Rare, autosomal dominant disease. PAPA syndrome is considered among them periodic fever syndromes / autoinflammatory diseases.

Diagnosis

  • Debut before 10's age
    • Pauciarical (four or less joints), destructive Arthritis
      • Elbows, knees, ankles
    • Significant acne problems in the teens
    • pyoderma- looked like sterile abscesses on the skin
  • Mutation in the PSTPIP1 gene

Treatment

Corticosteroids, TNF inhibitors (etanercept), IL-1 inhibitors (anakinra, canekinumab)

Literature

Auto-inflammatory diseases (BINDEVEVSSYKDOMMER.no)


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