Meige disease
| Classification according to ICD-10 | |
|---|---|
| Q82.0 | Hereditary lymphedema |
| ICD-10 online (WHO version 2019) | |
The Meige's disease is a very rare congenital form of a Lymphödemes with the main features of manifestation in the age of puberty and other congenital anomalies .
Synonyms are: Hereditary lymphedema type II Meige; Trophedema Meige; Lymphedema, Meige type; English familial lymphedema praecox; Late-onset lymphedema; Meige's disease
The disease should not be confused with Meige syndrome , a rare neurological disorder.
The name refers to the author of the description from 1889 by the French neurologist Henry Meige (1866–1940), a student of Jean-Martin Charcot .
The first description as lymphatic edema with malformations comes from Rudolf Virchow in 1863 .
distribution
The frequency is not known, the inheritance is autosomal dominant ; women are affected twice as often as men.
root cause
The cause is not yet known, there was a mutation in FOXC2 - Gen discussed but not operated.
Clinical manifestations
Clinical criteria are:
- Lymphedema of the lower extremities that occurs abruptly around puberty, often painful with acute signs of inflammation
- rarer lymphedema of the genitals, upper extremities, face or thorax with pleural effusions
- often partial membranous syndactyla of the toes
- double row eyelashes
- also myopia , ptosis , yellow nails, extradural cysts or vertebral anomalies, cleft palate , sinusitis and bronchiectasis
- Short stature , hypogenitalism , intrahepatic cholestasis
Sometimes there are diminished or absent lymph nodes in the armpit and above the [[inguinal ligament <inguinal ligament]].
history
The disease used to be known as Nun-Milroy-Meige Syndrome .
literature
- H. Meige: Le trophoedème chronique héréditaire. In: Nouvelle iconographie de la Salpêtrière , Paris, Vol. 12: 1889, pp. 453-480
Individual evidence
- ↑ a b c Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
- ↑ a b Meige disease. In: Orphanet (Rare Disease Database).
- ↑ H. Meige: dystrophy oedematose hereditaire. In: La presse médicale (Paris), Vol. 6, 1889, pp. 341-343.
- ^ Who named it Henry Meige
- ^ Who named it Nun-Milroy-Meige disease
- ↑ a b Encyclopedia Dermatology
- ↑ T. Rezaie, R. Ghoroghchian, R. Bell, G. Brice, A. Hasan, K. Burnand, S. Vernon, S. Mansour, P. Mortimer, S. Jeffery, A. Child, M. Sarfarazi: Primary non-syndromic lymphoedema (Meige disease) is not caused by mutations in FOXC2. In: European Journal of Human Genetics : EJHG. Vol. 16, No. 3, March 2008, pp. 300-304, doi: 10.1038 / sj.ejhg . 5201982 , PMID 18197197 .
- ^ Entry on Nonne-Milroy-Meige-Syndrome in Flexikon , a Wiki of the DocCheck company
