Abstract
Pyoderma gangrenosum, pyogenic sterile arthritis and acne (PAPA syndrome) is a hereditary, autosomal dominant, auto- inflammatory disease caused by missense mutation in the proline/serine/threonine phosphatase-interacting protein 1 (PSTPIP1) gene and predominantly affecting the joints and skin. Many reports now suggest that the clinical phenotype of PAPA syndrome may vary among people carrying the mutation and can lack the classic triad of pyogenic sterile arthritis, Pyoderma gangrenosum and cystic acne. No definite treatment strategy has been established for this PAPA like syndrome so far. We describe the treatment response of corticosteroids in a 23-year-old male with PAPA like syndrome and Takayasu arteritis, an association which has never been reported before.
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