P381 Transient periosteal hyperostosis with dysproteinemia (Goldbloom syndrome): two cases report Riccardo Papa, Alessandro Consolaro, Francesca Minoia, Roberta Caorsi, Gianmichele Magnano, Marco Gattorno, Angelo Ravelli, Paolo Picco Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy; Radiologia, Istituto Giannina Gaslini, Genoa, Italy Presenting author: Riccardo Papa Pediatric Rheumatology 2017, 15(Suppl 1):P381
Background PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum and acne) is a rare autosomal-dominant autoinflammatory disease caused by mutations in PSTPIP1gene. Typically presents with recurrent sterile, erosive arthritis in childhood, occurring spontaneously or after minor trauma, occasionally resulting in significant joint destruction. By puberty, joint symptoms tend to subside and cutaneous symptoms increase. Cutaneous manifestations include pathergy, frequently with abscesses at the sites of injections, severe cystic acne, and recurrent nonhealing sterile ulcers, often diagnosed as PG.
Kawasaki disease (KD) typically presents in children younger then 5y as a febrile illness with mucocutaneous changes. If untreated, KD can result in coronary aneurisms in 25% patients.
Catatonia is a rarely reviewed clinical feature of neuropsychiatric (NP) manifestation of pediatric systemic lupus erythematosus (pSLE). It is a state of neurogenic motor immobility, and behavioral abnormality manifested by stupor.
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