Lymphedema, type nonne-milroy Q82.0
Synonym(s)
elephantiasis congenita hereditaria; Lymphedema; Milroy's disease; Trophodema; Trophodema type Nonne-Milroy; Type Nonne-Milroy
History
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Nun, 1891; Milroy, 1892
Definition
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Genetically determined primary lymphedema. Probably irregular autosomal dominant inheritance, s.a. lymphedema, type Meige.
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Etiopathogenesis
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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.
Manifestation
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Especially with girls.
Localization
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Feet, lower legs, rarely hands and forearms.
Clinic
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Painless, initially compressible edema, later reactive tissue fibrosis.
Therapy
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Compression stockings on a trial basis.
Progression/forecast
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Chronic recurrent course, often worsening in puberty.
Literature
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- 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
Recommended articles
Incoming links (5)
Lymphatic malformations; Lymphedema, type meige; Milroy's disease; Trophodema; Trophodema, type nonne-milroy;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
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Prof. Dr. med. Peter Altmeyer
