Genetic disease
Primary lymphedema
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Cadherin EGF LAG seven-pass G-type receptor 1-related late-onset primary lymphedema (disorder)
CELSR1-related late-onset primary lymphedema
Cadherin EGF LAG seven-pass G-type receptor 1-related late-onset primary lymphedema
A rare genetic primary lymphedema characterized by unilateral or bilateral lower limb lymphedema of variable severity. The condition shows almost complete penetrance with onset in childhood or adolescence in females, whereas in males it shows incomplete penetrance with later onset of disease. Lymphoscintigraphy in more severely affected individuals reveals lymphatic abnormalities consistent with lymphangiectasia, valve dysfunction, and thoracic duct reflux.
Id1222668001
StatusPrimitive
Associated morphologyLymphatic edema
Finding siteLimb structure
Pathological processPathological developmental process
ICD-10 complex map reference set
TargetI89.0
RuleTRUE
AdviceALWAYS I89.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified