Abstract
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.
Highlights
PAPA syndrome is a rare autosomal-dominant autoinflammatory disease caused by mutations in PSTPIP1gene
To report a case of hepatitis B infection revealed with interleukin-1 receptor antagonist for PAPA syndrome
We reported a boy with genetically confirmed PAPA syndrome
Summary
V Selmanovic1*, F DeBenedeti[2], A Omercahić-Dizdarevic[1], E Kovac-Vidakovic[3], S Mehanic[4], A Cengic[1]. From 8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases Dresden, Germany. From 8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases Dresden, Germany. 30 September - 3 October 2015
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