|
autosomaal recessieve ichthyosis exfoliativa (aandoening)
autosomaal recessieve ichthyosis exfoliativa
autosomaal recessieve exfoliatieve ichtyose
autosomaal recessieve ichthyosis bullosa van Siemens
Autosomal recessive exfoliative ichthyosis
Ichthyosis exfoliativa
Exfoliative ichthyosis
An inherited non-syndromic congenital ichthyosis characterized by the infancy-onset of palmoplantar peeling of the skin (aggravated by exposure to water and by occlusion) associated with dry, scaly skin over most of the body. Pruritus and hypohidrosis may also be associated. Well-demarcated areas of denuded skin appear in moist and traumatized regions and skin biopsies reveal reduced cell-cell adhesion in the basal and suprabasal layers, prominent intercellular edema, numerous aggregates of keratin filaments in basal keratinocytes, attenuated cornified cell envelopes, and epidermal barrier impairment.
Id763768001
StatusPrimitive
Associated morphologyhyperkeratose
Finding sitegehele huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Has interpretationafwijkend
Interpretskeratinisatie
referentieset met complexe 'mapping' naar ICD-10
TargetQ80.8
RuleTRUE
AdviceALWAYS Q80.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified