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neonatale inflammatoire huid- en darmziekte (aandoening)
neonatale inflammatoire huid- en darmziekte
Neonatal inflammatory skin and bowel disease
A rare life-threatening autoinflammatory syndrome with immune deficiency disorder characterized by early-onset life-long inflammation affecting the skin and bowel associated with recurrent infections. Presents with perioral and perianal psoriasiform erythema and papular eruption with pustules, failure to thrive associated with chronic malabsorptive diarrhea, intercurrent gastrointestinal infections and feeding troubles, as well as absent, short or broken hair and trichomegaly. Recurrent cutaneous and pulmonary infections lead to recurrent blepharitis, otitis externa and bronchiolitis.
Id773662009
StatusPrimitive
Associated morphologychronische ontsteking
Finding sitestructuur van intestinum
Occurrenceneonataal
Associated morphologychronische ontsteking
Finding sitestructuur van huid
Occurrenceneonataal
Clinical coursechronisch
Pathological processafwijkend immuunproces
referentieset met complexe 'mapping' naar ICD-10
TargetD89.9
RuleTRUE
AdviceALWAYS D89.9 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified
TargetL08.8
RuleTRUE
AdviceALWAYS L08.8
CorrelationSNOMED CT source code to target map code correlation not specified
TargetK52.9
RuleTRUE
AdviceALWAYS K52.9
CorrelationSNOMED CT source code to target map code correlation not specified