primary lymphedema
MONDO:0019175Mondo
Findings
No curated finding names primary lymphedema yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A congenital condition that results in swelling in the arms or legs, and can occur during adolescence or adulthood. Loss of motion and pain may also accompany the swelling. Protein-rich lymphatic fluid accumulates in tissues, engorging and enlarging vessels and often causing visible swelling, tenderness, and pain. Left untreated, the affected tissues may continue to swell, and can become hardened or fibrotic and susceptible to infection.
Definition from the Mondo Disease Ontology (MONDO:0019175), read 2026-09-29. CC BY 4.0.
Where it sits
- A kind of
- Narrower terms (13)
- Aagenaes syndrome
- aplasia cutis congenita-intestinal lymphangiectasia syndrome
- CELSR1-related late-onset primary lymphedema
- chylous ascites
- congenital pulmonary lymphangiectasia
- EPHB4-related lymphatic-related hydrops fetalis
- German syndrome
- GJC2-related late-onset primary lymphedema
- hypotrichosis-lymphedema-telangiectasia syndrome (grouping)
- lymphatic malformation
- lymphedema-atrial septal defects-facial changes syndrome
- mullerian derivatives-lymphangiectasia-polydactyly syndrome
- warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome
Other names
1 name
Resolves to: primary lymphedema
- Also called
- Troncular lymphatic malformation