Nonne-Milroy-Meige disease

Chronic familial lymphoedema of the limbs, which manifests with pittings and brawny swellings (lymphoedema) of the ankles and shins. Often associated with inflammation and various defects, including distichiasis, extradural cysts, vertebral anomalies, cerebrovascular malformations, yellow nails, and sensorineural hearing loss. Congenital or onset in first or second decade of life. It is due to inadequate lymph flow through an anatomically abnormal lymphatic system. Prevalent in females (70-80%). Pain, sometimes severe, is a common feature. Autosomal dominant inheritance with variable expressivity.

There is a lot of confusion about the correct term for this condition. According to some authors the term Nonne-Milroy-Meige syndrome is obsolete, as this is really two distinct entities, Hereditary lymphedema I (Nonne-Milroy syndrome) with onset at birth to 35 years of age; and hereditary lymphedema type II (Meige syndrome) with onset after 35 years. The eponym Milroy disease was given to it by Sir William Osler in his textbook The Principles and Practice of Medicine (New York, 1892). In France the disorder is sometimes called Meige Disease, in Germany Nonne-Milroy-Meige.

Hereditary lymphedema of the legs was described by Nonne in 1891. In 1892 Milroy described a clergyman aged 31 years who had returned from missionary work in India and had requested an examination for life insurance purposes. This person had oedema in the lower legs which had been present all his life and his mother had been similarly affected. A few years previously a relative had produced a history of the family in the USA covering a 250-year period. From this information Milroy was able to identify 22 persons with the condition in six generations and to determine that the disorder had entered the kindred by marriage in 1768. Meige described the condition in 1898. However, the condition was first described by Virchow in 1863 as «Lymphatisches Ödem mit Missbildungen».

We thank Ouidga Meyers for correcting an error in our original entry.

• M. Nonne:
  Vier Fälle von Elephantiasis congenita hereditaria.
  [Virchows] Archiv für pathologische Anatomie und Physiologie und für klinische Medicin, Berlin, 1891, 189-196.
• W. F. Milroy:
  An undescribed variety of herditary edema.
  New York Medical Journal, 1892, 56: 505-508. Chronic hereditary oedema: Milroy's disease.
  Journal of the American Medical Association, Chicago, 1928, 91: 1172.
• H. Meige:
  Dystrophie oedematose hereditaire.
  La presse médicale, Paris, 1898, 6: 341-343. Le trophoedème chronique héréditaire.
  Nouvelle iconographie de la Salpêtrière, Paris, 1899, 12: 453-480.