PAPA syndrome (pyogenische arthritis, pyoderma gangrenosum and acne) is an autosomal dominant genetic disease affects the skin and joints.
PAPA syndrome is caused by mutations in a gene on chromosome 15q. The mutant gene produces a hyperphosphorylated protein that binds strongly to Pyrin and thus inhibits the anti-inflammatory activity of the Pyrins.
PAPA syndrome (pyogenische arthritis, pyoderma gangrenosum and acne) is an autosomal dominant genetic disease affects the skin and joints. PAPA syndrome is caused by mutations in a gene on chromosome 15q. The mutant gene produces a hyperphosphorylated protein that binds strongly to Pyrin and thus inhibits the anti-inflammatory activity of the Pyrins. Arthritis begins in the first decade of life and is increasingly destructive. Episodes of a slight trauma can trigger arthritis. Poorly healing ulcers with blurry edge occur, often at sites of injury (eg. As Impfeinstichen). An acne is usually with nodules and cysts inflammatory and leaves scars if not treated. PAPA syndrome © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/404_pyoderma_gangrenosum_slide_20_springer_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / ? professional / images / 404_pyoderma_gangrenosum_slide_20_springer_high_de.jpg lang = en & thn = 0 ‘, title:’ PAPA syndrome ‘, description:’ u003Ca id = “v37897727 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003ePyoderma gangrenosum (necrosis with deep ulceration) and nodulocystic acne are characteristic of a PAPA syndrome u003c / p u003e u003c / div u003e ‘credits’. © Springer Science + Business Media ‘