Developmental disorder
Lymphedema of limb
||
Primary lymphedema (disorder)
Primary lymphedema
Primary lymphatic edema
A lymphatic system malformation with characteristics of swelling of an extremity that can be associated with other lymphatic effusions, due to an underlying developmental anomaly of the lymphatic system (abnormal lymphangiogenesis). It can be hereditary or not and be congenital or late onset.
Id1217009002
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
|
CELSR1-related late-onset primary lymphedema
Congenital primary lymphedema of Gordon
EPHB4-related lymphatic-related hydrops fetalis
GJC2-related late-onset primary lymphedema
Hereditary lymphedema20
PIEZO1-related generalized lymphatic dysplasia with non-immune hydrops fetalis
Primary lymphedema with systemic involvement1
Primary lymphedema with visceral involvement1
Syndromic primary lymphedema