Preferred Label : Pyogenic arthritis - pyoderma gangrenosum - acne;
ICD-11 definition : Pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is a rare
pleiotropic autoinflammatory disorder of childhood, primarily affecting the joints
and skin, characterised by pauciarticular, nonaxial, sometimes recurrent, sterile
arthritis begining in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence
and beyond.;
ICD-11 synonym : PAPA - [pyogenic arthritis, pyoderma gangrenosum and acne] syndrome; PAPA syndrome;
Origin ID : 1113124609;
Currated CISMeF NLP mapping
Pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is a rare
pleiotropic autoinflammatory disorder of childhood, primarily affecting the joints
and skin, characterised by pauciarticular, nonaxial, sometimes recurrent, sterile
arthritis begining in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence
and beyond.
