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syndroom van 'peeling skin', leukonychie, acrale keratosis punctata, cheilitis en 'knuckle pads' (aandoening)
syndroom van 'peeling skin', leukonychie, acrale keratosis punctata, cheilitis en 'knuckle pads'
PLACK-syndroom
syndroom van afschilfering van huid, leuconychia, acrale puntvormige keratose, cheilitis en tylositas articuli
syndroom van vervellende huid, leukonychie, acrale puntvormige keratose, cheilitis en knokkelkussentjes
PLACK syndrome
PLACK (peeling skin, leukonychia, acral punctate keratoses, cheilitis, knuckle pads) syndrome
Peeling skin, leukonychia, acral punctate keratoses, cheilitis, knuckle pads syndrome
Peeling skin, leuconychia, acral punctate keratoses, cheilitis, knuckle pads syndrome
A rare genetic skin disease characterized by generalized skin peeling, leukonychia, acral punctate keratoses coalescing into focal keratoderma on the weight-bearing areas, angular cheilitis and knuckle pads with multiple hyperkeratotic micropapules. The skin appears dry and scaly with superficial exfoliation and underlying erythema. Histopathologic examination of affected skin areas shows hyperkeratosis, acanthosis and intraepidermal clefting with irregular acantholysis. Additional systemic abnormalities are absent.
Id1237509001
StatusPrimitive
Has interpretationafwijkend
Interpretskeratinisatie
Interpretskleur van nagel
Associated morphologyexfoliatieve laesie
Finding sitestructuur van huid
Associated morphologymorfologische afwijking
Finding sitestructuur van nagel
SNOMED CT to Orphanet simple map444138
SNOMED CT to ICD-10 extended map
TargetQ82.8
RuleTRUE
AdviceALWAYS Q82.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified