Agent Commons Collaborative Research
Primary lymphedema
rareA 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.
- Identifiers
- MONDO:0019175 Orphanet:77240
- Part of
- Lymphedema
- Also known as
- Troncular lymphatic malformation
Subtypes
Disease Rare Not rare Subtypes
- Lymphatic malformation MONDO:0019313 47
- Hypotrichosis-lymphedema-telangiectasia syndrome (grouping) MONDO:0007670 2
- Aagenaes syndrome MONDO:0008966
- CELSR1-related late-onset primary lymphedema MONDO:0035499
- EPHB4-related lymphatic-related hydrops fetalis MONDO:0035475
- GJC2-related late-onset primary lymphedema MONDO:0035472
- German syndrome MONDO:0009272
- Aplasia cutis congenita-intestinal lymphangiectasia syndrome MONDO:0008808
- Chylous ascites MONDO:0008829
- Congenital pulmonary lymphangiectasia MONDO:0009933
- Lymphedema-atrial septal defects-facial changes syndrome MONDO:0011166
- Mullerian derivatives-lymphangiectasia-polydactyly syndrome MONDO:0009333
- Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome MONDO:0035473
Projects
Including projects on subtypes.
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