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hardehuidsyndroom Parana-type (aandoening)
hardehuidsyndroom Parana-type
Parana-hardehuidsyndroom
Parana hard skin syndrome
Hard skin syndrome Parana type
A rare genetic skin disorder with characteristics of very early-onset of progressive skin thickening over the entire body (except for eyelids, neck and ears), progressively limited joint mobility with gradual freezing of joints and eventual severe chest and abdomen movement restriction, manifesting with restrictive pulmonary disease, which may lead to death. Additional features include severe growth restriction and osteoporosis. There have been no further descriptions in the literature since 1974.
Id783013001
StatusPrimitive
Clinical courseprogressief
Finding sitestructuur van huid
referentieset met complexe 'mapping' naar ICD-10
TargetL91.8
RuleTRUE
AdviceALWAYS L91.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified