acne cystica	artritis	auto-inflammatoire aandoening	autosomaal dominante hereditaire aandoening	hereditaire aandoening van bewegingsapparaat	hereditaire aandoening van immuunsysteem	hereditaire aandoening van integumentum	primaire immunodeficiëntie	pyoderma gangraenosum
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syndroom van pyogene artritis, pyoderma gangraenosum en acne (aandoening)
	syndroom van pyogene artritis, pyoderma gangraenosum en acne
	PAPA-syndroom
	Pyogenic arthritis, pyoderma gangrenosum, acne syndrome
	PAPA syndrome PAPA (pyogenic arthritis, pyoderma gangrenosum, acne) syndrome Familial recurrent arthritis
	A rare pleiotropic auto-inflammatory disorder of childhood, primarily affecting the joints and skin. The first affected family contained ten affected members from three generations and manifested variable expression of a pauciarticular, nonaxial, arthritis that began in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence and beyond. Recurrent sterile arthritis usually occurs after minor trauma, but can also occur spontaneously. PAPA syndrome is a self-limiting disease, but it can lead to severe joint destruction. The gene responsible for the syndrome is the proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1).

Id	724015007
Status	Primitive

Associated morphology	neutrofiele infiltratie
Finding site	structuur van huid

Associated morphology	cysteuze ontsteking
Finding site	complex van haar, haarfollikel, musculus arrector pili en talgklier

Associated morphology	purulente ontsteking
Finding site	structuur van huid

Associated morphology	purulente ontsteking
Finding site	structuur van gewricht
Occurrence	kinderleeftijd

Associated morphology	inflammatoire morfologie
Finding site	structuur van immuunsysteem
Pathological process	afwijkend immuunproces

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	L70.9
Term	Acne, niet gespecificeerd

Target	L88
Term	Pyoderma gangraenosum

Target	M00.99
Term	Pyogene artritis, niet gespecificeerd van lokalisatie niet gespecificeerd

Target	Z84.8
Term	Familie-anamnese met overige gespecificeerde aandoeningen

SNOMED CT to Orphanet simple map

69126

SNOMED CT to ICD-10 extended map

Target	M00.99
Rule	TRUE
Advice	ALWAYS M00.99 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified

Target	L88
Rule	TRUE
Advice	ALWAYS L88
Correlation	SNOMED CT source code to target map code correlation not specified

Target	L70.0
Rule	TRUE
Advice	ALWAYS L70.0
Correlation	SNOMED CT source code to target map code correlation not specified