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syndroom van segmentale overgroei, lipomatose, arterioveneuze malformatie en epidermale naevus (aandoening)
syndroom van segmentale overgroei, lipomatose, arterioveneuze malformatie en epidermale naevus
syndroom van segmentale uitloper, lipomatose, arterioveneuze malformatie en epidermale naevus
SOLAMEN-syndroom
Segmental outgrowth, lipomatosis, arteriovenous malformation, epidermal nevus syndrome
SOLAMEN syndrome
A rare genetic, poly malformative syndrome with characteristics of progressive, proportionate, asymmetric segmental overgrowth (with soft tissue hypertrophy and ballooning effect) that develops and progresses rapidly in early childhood, arteriovenous and lymphatic vascular malformations, lipomatosis and linear epidermal nevus (arranged in whorls along the lines of Blaschko). Clinical symptoms of Cowden syndrome, such as macrocephaly and progressive development of numerous hypertrophic hamartomatous and neoplastic lesions involving multiple organs and systems are also associated. Patients present an increased risk of developing cancer.
Id763867001
StatusPrimitive
Associated morphologymorfologische afwijking
Finding sitestructuur van bloedvat
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologyepidermale naevus
Finding sitestructuur van huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologylipomatose
Occurrencecongenitaal
referentieset met complexe 'mapping' naar ICD-10
TargetQ85.8
RuleTRUE
AdviceALWAYS Q85.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified