Lymphedema, primaryQ82.0

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

chronic hereditary; congenital; elephantiasis congenita hereditaria; hereditary; hereditary congenital lymphedema; hereditary lymphedema; Lymphedema; Meige-Nonne-Milroy syndrome; Nonne-Milroy-Meige syndrome; Trophodema; Trophödem chronic hereditary; Trophoedème chronique héréditaire

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HistoryThis section has been translated automatically.

Nun, 1891; Milroy, 1892; Meige, 1898

DefinitionThis section has been translated automatically.

Hereditary malformation (local aplasia or dysplasia) of the lymphatic system, which is caused on the one hand by mutations of individual genes, and on the other hand by chromosomal aberrations (anomalies in the structure or number of chromosomes).

In the case of chromosomal aberrations, syndromal clinical pictures with malformations in several organ systems in combination with lymphedema are found.

Lymphoedema can be described as hereditary if the transition of the mutated gene from parents to children can be traced.

Mutations can also occur spontaneously during somatic development. In these cases one speaks of sporadic (congenital) primary lymphedema

ClassificationThis section has been translated automatically.

S.u. lymphedema.

Occurrence/EpidemiologyThis section has been translated automatically.

About 35-40% of the lymphedema clientele suffers from primary lymphedema.

EtiopathogenesisThis section has been translated automatically.

Etiological classification:
  • Hypoplasia or aplasia of the lymph vessels
  • Lymphangiectasia (dilation of the vessels leading to valvular hypoplasia)
  • (primary) lymph node fibrosis, an embryonic malformation of lymph nodes with intact lymph vessels.

ManifestationThis section has been translated automatically.

Women are affected 5-6 times more frequently than men.

LocalizationThis section has been translated automatically.

Lower extremity

DiagnosisThis section has been translated automatically.

Clinical picture is diagnostic.

Complication(s)This section has been translated automatically.

Chronic erysipelas, therapy-resistant ulcus cruris, increasing physical immobility with consecutive obesity; elephantiasis nostras.

TherapyThis section has been translated automatically.

Only symptomatic therapy possible. Lymph drainage and lymph massage. Trial with compression bandages or compression stockings of higher compression classes (III and IV), if possible custom-made adjustment. If necessary, additional attempt at plastic surgery measures, see lymphedema below.

LiteratureThis section has been translated automatically.

  1. Meige H (1898) Dystrophy oedemateuse hereditaire. Press Med 6: 341-343
  2. Meige H (1899) Le trophoedème chronique héréditaire. Nouvelle iconographie de la Salpêtrière (Paris) 12: 453-480
  3. Milroy WF (1892) An undescribed variety of hereditary edema. New York Medical Journal 56: 505-508
  4. Milroy WF (1928) Chronic hereditary edema: Milroy's disease. JAMA 91: 1172-1175
  5. Nun M (1891) Four cases of elephantiasis congenita hereditaria. Virchows Arch pathol Anat Physiol klin Med (Berlin) 189-196

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer