
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
- 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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