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DEAR EDITOR, Mutations in the PSTPIP1 gene, encoding prolineserine-threonine phosphatase-interactive protein 1, were first identified in an autosomal dominant syndrome called PAPA, associated with pyogenic sterile arthritis, pyoderma gangrenosum (PG) and cystic acne. 1,2We report a patient with an autoinflammatory syndrome called PSTPIP1-associated myeloid-related proteinaemia inflammatory (PAMI) syndrome. 3 A 23-year-old man had a 3-year-history of skin ulcerations. During childhood he exhibited anaemia (81 g L À1 ), neutropenia (0.3 g L À1 ), mild thrombocytopenia and hepatosplenomegaly. Presence of anaemia and of recurrent infections prompted a bone marrow biopsy, which showed evidence for disturbed myelopoiesis beyond the promyelocyte-myelocyte level. From the age of 7 years he developed relapsing sterile osteomyelitis affecting the left talus, tibia and fibula. In his adolescence he had relapsing sterile arthritis of one knee and elbow.

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