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syndroom van palmoplantaire hyperkeratose, XX-geslachtsomkering en predispositie voor plaveiselcelcarcinoom (aandoening)
syndroom van palmoplantaire hyperkeratose, XX-geslachtsomkering en predispositie voor plaveiselcelcarcinoom
palmoplantaire keratodermie-XX-geslachtsomkering-predispositie voor plaveiselcelcarcinoom-syndroom
Palmoplantar keratoderma, 46,XX sex reversal, predisposition to squamous cell carcinoma syndrome
This syndrome has characteristics of sex reversal in males with a 46, XX (SRY-negative) karyotype, palmoplantar hyperkeratosis and a predisposition to squamous cell carcinoma. To date, five cases (four of whom were brothers) have been described.
Id722202006
StatusPrimitive
Associated morphologymorfologische afwijking
Finding sitestructuur van gonade
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologyhyperkeratose
Finding sitestructuur van huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
referentieset met complexe 'mapping' naar ICD-10
TargetQ56.0
RuleTRUE
AdviceALWAYS Q56.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified