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familiaire progressieve hyperpigmentatie en hypopigmentatie (aandoening)
familiaire progressieve hyperpigmentatie en hypopigmentatie
FPHH
Familial progressive hyper and hypopigmentation
Familial progressive hyperpigmentation and hypopigmentation of skin
Familial progressive hyper- and hypopigmentation is a rare, genetic, skin pigmentation anomaly disorder characterized by progressive, diffuse, partly blotchy, hyperpigmented lesions that are intermixed with multiple café-au-lait spots, hypopigmented maculae and lentigines and are located on the face, neck, trunk and limbs, as well as, frequently, the palms, soles and oral mucosa. Dyspigmentation pattern can range from well isolated café-au-lait/hypopigmented patches on a background of normal-appearing skin to confetti-like or mottled appearance.
Id763368004
StatusPrimitive
Associated morphologypigmentverschuiving
Finding sitestructuur van huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetL81.8
TermOverige gespecificeerde pigmentatiestoornissen
SNOMED CT to Orphanet simple map280628
SNOMED CT to ICD-10 extended map
TargetL81.8
RuleTRUE
AdviceALWAYS L81.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified