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Start free with EleplanPrimary lymphedema
ORPHA:77240Category
What it is
Primary lymphedema is a lymphatic system malformation characterized by 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.
Key facts
- Prevalence
- 1-5 / 10 000 (Europe)
- Age of onset
- All ages
- Inheritance
- Autosomal dominant, Autosomal recessive
- Classified as
- Category
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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