Nun-Milroy-Meige Syndrome

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The autosomal dominant inheritance
Classification according to ICD-10
Q82.0 Hereditary lymphedema
ICD-10 online (WHO version 2019)

The nun-Milroy-Meige syndrome describes a familial ( autosomal -dominant inherited ), chronic , painless lymphedema of the lower and upper extremities, in rare cases. It results in short stature , acromicias , obesity (breeches type), hypogonadism and mental retardation . Those affected are still prone to ulcers and are prone to secondary infections .

The syndrome is named after the German neurologist Max Nonne (1861–1959), the American internist William Forsyth Milroy (1855–1942) and the French internist Henry Meige (1866–1940).

The term is more of historical significance today and has been replaced by other names, as the clinical picture can easily be divided clinically into two separate entities :

  • Hereditary lymphedema type 1 ( Nun-Milroy syndrome ), which does not result in any further malformations and is congenital
  • Hereditary lymphedema type 2 ( Meige syndrome ), which is associated with malformations and manifests itself around the period of puberty .

swell

  • Roche Lexicon Medicine, 5th edition, Urban & Fischer, September 2003.

Individual evidence

  1. ^ Entry on Nonne-Milroy-Meige-Syndrome in Flexikon , a Wiki of the DocCheck company
  2. Milroy disease. In: Orphanet (Rare Disease Database).
  3. ^ Meige disease. In: Orphanet (Rare Disease Database).