Medical Subject Headings

Last uploaded: March 22, 2026

Id	http://purl.bioontology.org/ontology/MESH/C536253 http://purl.bioontology.org/ontology/MESH/C536253
Preferred Name	Pyogenic arthritis, pyoderma gangrenosum, and acne
Synonyms	Familial recurrent arthritis Pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne PAPA syndrome Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne
Type	http://www.w3.org/2002/07/owl#Class

altLabel	Familial recurrent arthritis Pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne PAPA syndrome Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne
prefLabel	Pyogenic arthritis, pyoderma gangrenosum, and acne
Scope Statement	A hereditary autosomal dominant disorder characterized by pyogenic (infectious) arthritis, pyoderma gangrenosum, and severe cystic acne. Onset is in childhood; pyoderma gangrenosum; and severe cystic acne occur in adolescence and beyond. Other less common features may include adult-onset DIABETES MELLITUS, TYPE I; PROTEINURIA, ABSCESS formation at the site of parenteral injections, and cytopenias attributable to SULFONAMIDE medications. Mutations in the PSTPIP1 gene have been identified. OMIM: 604416
type	http://www.w3.org/2002/07/owl#Class
tui	T047
HM	D000152 D001170 D017511
SC	3
TERMUI	T000883562 T738747 T826248 T738743 T738745
TH	OMIM (2013) NLM (2013) ORD (2010)
MMR	20150818
notation	C536253
Semantic type UMLS property	http://purl.bioontology.org/ontology/STY/T047
cui	C1858361
MeSH Frequency	61
DSC	20100825
Inverse of RB	0
Mapped to	http://purl.bioontology.org/ontology/MESH/D000152 http://purl.bioontology.org/ontology/MESH/D001170 http://purl.bioontology.org/ontology/MESH/D017511
MDA	20100825

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