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sindrome papa

Pyogenic arthritis-pyoderma gangrenosum-acne syndrome is a rare pleiotropic autoinflammatory disorder of childhood, primarily affecting the joints and skin.

        sindrome papa
        1
        TNFa blockade appears to be effective in treating the cutaneous manifestations of PAPA syndrome.
        Pubmed
        2
        Neutrophil granule proteins are markedly elevated ex vivo and in the plasma, and elevated levels might be compatible with a diagnosis of PAPA syndrome.
        Pubmed
        3
        Conclusion: This analysis of 5 patients demonstrates that mutations in PSTPIP1 are incompletely penetrant and variably expressed in the PAPA syndrome.
        Pubmed