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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).
Id724015007
StatusPrimitive
Associated morphologyinfiltratie
Finding sitestructuur van huid
Associated morphologypurulente ontsteking
Finding sitestructuur van huid
Associated morphologypurulente ontsteking
Finding sitestructuur van gewricht
Occurrencekinderleeftijd
Associated morphologyinflammatoire morfologie
Finding sitestructuur van immuunsysteem
Pathological processafwijkend immuunproces
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetL70.9
TermAcne, niet gespecificeerd
TargetL88
TermPyoderma gangraenosum
TargetM00.99
TermPyogene artritis, niet gespecificeerd van lokalisatie niet gespecificeerd
TargetZ84.8
TermFamilie-anamnese met overige gespecificeerde aandoeningen
ICD-10 complex map reference set
TargetM00.99
RuleTRUE
AdviceALWAYS M00.99 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified
TargetL88
RuleTRUE
AdviceALWAYS L88
CorrelationSNOMED CT source code to target map code correlation not specified
TargetL70.0
RuleTRUE
AdviceALWAYS L70.0
CorrelationSNOMED CT source code to target map code correlation not specified