Genetic disease
Primary lymphedema
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Gap junction protein gamma 2-related late-onset primary lymphedema (disorder)
GJC2-related late-onset primary lymphedema
Gap junction protein gamma 2-related late-onset primary lymphedema
A rare genetic primary lymphedema characterized by lymphedema of all four limbs with age of onset ranging from birth to adulthood. Manifestations are of variable severity, and upper limb involvement may develop only later in the disease course. Recurrent episodes of cellulitis and skin infections are observed in severe cases. Varicose veins and venous incompetence have been reported in association.
Id1222670005
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