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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 gewricht
Occurrencekinderleeftijd
Pathological processauto-immuunproces
Associated morphologypurulente ontsteking
Finding sitestructuur van huid
Occurrencekinderleeftijd
Pathological processauto-immuunproces
referentieset met complexe 'mapping' naar ICD-10
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