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Lymphedema, type nonne-milroyQ82.0
Synonym(s)
elephantiasis congenita hereditaria; Lymphedema; Milroy's disease; Trophodema; Trophodema type Nonne-Milroy; Type Nonne-Milroy
HistoryThis section has been translated automatically.
Nun, 1891; Milroy, 1892
DefinitionThis section has been translated automatically.
Genetically determined primary lymphedema. Probably irregular autosomal dominant inheritance, s.a. lymphedema, type Meige.
EtiopathogenesisThis section has been translated automatically.
The cause is a mutation of the FLT4 gene, which is mapped on chromosome 5q35.3. The mutated gene product of the FLT4 gene is the vascular endothelial growth factor.
ManifestationThis section has been translated automatically.
Especially with girls.
LocalizationThis section has been translated automatically.
Feet, lower legs, rarely hands and forearms.
Clinical featuresThis section has been translated automatically.
Painless, initially compressible edema, later reactive tissue fibrosis.
TherapyThis section has been translated automatically.
Compression stockings on a trial basis.
Progression/forecastThis section has been translated automatically.
Chronic recurrent course, often worsening in puberty.
LiteratureThis section has been translated automatically.
- Garg BK (1974) Congenital lymphoedema. A report of 10 cases in a family. Indian J Pediat 41: 309-313
- Ho WL et al (2003) Radiological features of late-onset lymphoedema in Noonan's syndrome. Pediatric radiol 33: 200-202
- Meinecke P (1987) Letter to the editor: A genetic association between microcephaly and lymphedema. At J Med Genet 26: 233.
- Milroy WF (1892) An undescribed variety of hereditary edema. N Y Med J 56: 505-508
- Mosquito J, Hoepffner W, Scheerschmidt G et al (1986) Early onset lymphoedema, recessive form - a new form of genetic lymphoedema syndrome. Eur J Pediatr 145: 195-198
- Nun M (1891) Four cases of elephantiasis congenita hereditaria. Arch Path Anat (Berlin) 125: 189-196