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hereditaire Behçet-achtige ziekte op kinderleeftijd (aandoening)
hereditaire Behçet-achtige ziekte op kinderleeftijd
Behçet-achtige ziekte door HA20
erfelijke pediatrische Behçet-achtige ziekte
op oculo-urogenitaal syndroom lijkende ziekte door haplo-insufficiëntie van A20
Hereditary pediatric Behçet-like disease
Behçet-like disease due to HA20
Behçet-like disease due to haploinsufficiency of A20
A rare autosomal dominant autoinflammatory syndrome characterized by early onset systemic inflammation with autoimmune manifestations and more rarely, humoral immune deficiency and increased production of circulating proinflammatory cytokines. Variable manifestations include recurrent oral aphthous ulcers, genital ulcers, arthralgia or arthritis, periodic fever, uveitis and severe gastrointestinal involvement (pain, diarrhea, vomiting, rectal bleeding).
Id1187119002
StatusPrimitive
Clinical courserecidiverend
Associated morphologyinflammatoire morfologie
ICD-10 complex map reference set
TargetD89.8
RuleTRUE
AdviceALWAYS D89.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified