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poikilodermie met neutropenie (aandoening)
poikilodermie met neutropenie
Poikiloderma with neutropenia
Poikiloderma with neutropenia Clericuzio type
Poikiloderma with neutropenia is a rare, genetic hereditary poikiloderma disorder characterized by early-onset poikiloderma (which typically begins in the extremities, progresses centripetally and eventually involves the trunk, face and ears) associated with chronic neutropenia, recurrent infections, pachyonychia and palmoplantar keratoderma. Growth and/or developmental delay and hepato- and/or splenomegaly are additional reported features.
Id772126000
StatusPrimitive
Associated morphologypoikilodermie
Finding sitestructuur van huid
Clinical coursechronisch
Pathological processafwijkend immuunproces
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetD82.8
TermImmunodeficiƫntie samengaand met andere gespecificeerde belangrijke defecten
SNOMED CT to Orphanet simple map221046
SNOMED CT to ICD-10 extended map
TargetD82.8
RuleTRUE
AdviceALWAYS D82.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified