Lymphedema (overview) I89.00

Due to its etiopathogenesis, lymphedema is divided into primary lymphedema (rare, mostly genetic) and non-genetic (acquired) secondary lymphedema. The group of non-classifiable, idiopathic lymphedema is listed in the group of genetic lymphedema.

Primary lymphedema: mainly in younger patients. Genetic or sporadic, unilateral or bilateral lymphedema manifesting at birth or in early adolescence due to structural lymphatic vessel abnormalities.

Primary lymphedema can be divided into genetic and idiopathic lymphedema.

• Genetic lymphedema:
  • Lymphoedema, type nun-milroy
  • Lymphedema, type Meige (corresponding to lymphedema, type Nonne-Milroy with other associated symptoms)
  • Lymphedema distichiasis syndrome
  • Yellow-nail syndrome
  • Noonan syndrome.
  • Lymphedema and other hereditary diseases (very rare):
    • Aagenaes Syndrome
    • Aarskog-Scott Syndrome
    • Buschke-Ollendorf syndrome
    • Lymphangiectasia-lymphedema syndrome (Hennekam syndrome)
    • Hypotrichosis-lymphedema-teleangiectasia syndrome
    • Kanzaki disease
    • OLEDAID syndrome.
  • Unclassified lymphedema:
    • Idiopathic lymphedema.
• Secondary lymphedema: Especially in elderly people. Acquired, unilateral or bilateral lymphedema due to acquired disturbances of lymph transport.

Genetic, inflammatory or caused by organ insufficiencies. Aetiopathogenetically, the disease is based on an imbalance between lymphatic filtration and the transport capacity of the lymphatic vessels. A distinction is made between the relative imbalance due to impairment of the lymphatic flow(afterload impairment) and the absolute imbalance due to increased filtration (preload impairment) (Wollina U et al. 2018). In the lymphostatic congestion area, congested plasma proteins cause a cellular reaction (macrophages, fibroblasts) and connective tissue proliferation with proliferation of blood vessels. Increased susceptibility to infection in the lymphostatic area can lead to recurrent erysipelas, which in turn cause secondary lymphedema.

Extent and heaviness of extremities, thickening of dermis (based on lymphostatic fibrosclerosis) and subcutaneous fatty tissue, verruciform epidermal hyperplasia (papillomatosis lymphostatica), post-inflammatory hyperpigmentation, onychodystrophy, onychogrypose, disturbance of nail growth Development of elephantiasis possible, see table 1.

• Anamnesis, clinic, (local findings, skin fold test/ Malleese sign), laboratory.
• In lymphedema of the lower extremity: Doppler or colour duplex to exclude the involvement of the veins.
• Extended diagnostics: Indirect lymphography, lymph scintigraphy; if necessary X-ray examination of the thorax, sonography of the abdomen, thorax, neck (lymph node enlargements, tumours?), computer tomography of the abdomen and the upper thoracic aperture; in rare, unclear cases magnetic resonance imaging (in lymphoedema: honeycomb pattern in the subcutis).

• Recurrent local arterial and mycotic infections
• Formation of elastolytic giant cell granulomas
• Combination with immunodeficiencies
• lymphatic cyst formation
• Lymphorrhoea
• Development of benign and malignant angiomatous tumors ( lymphangiosarcoma, spindle cell hemangioma).

• The focus is on consistently decongestive therapy!
• Földish 2-phase treatment:
• Initial: Deedematisation with manual and, if necessary, additional mechanical lymph drainage, compression bandage with short-stretch bandages, elevation of the limb.
• After no further decongestion can be achieved, the conservative phase follows: adjustment of the compression stocking or Pütter bandages. See also compression therapy below.
• Stabilisation measures under outpatient conditions: after maximum decongestion, prescription and consistent wearing of compression stockings (class III). Made to measure. Fitting in the morning in a slim leg condition; if necessary, incorporation of pads pre- or retromalleolar. Continuation of manual lymphatic drainage, if necessary additional intermittent mechanical compression. Coordination of a long-term treatment plan with patient and family, specific sports activities (cycling, hiking, swimming), treatment of obesity.

  Remember!

  Putting 2 pairs of compression stockings of compression stockings class II on top of each other corresponds to pressure class III!

Notice! The sole use of intermittent lymphatic drainage equipment (see below, pneumatic intermittent compression) is not recommended. It is only useful as a supplement to manual lymphatic drainage! Beforehand, apply to your health insurance company for the prescription of an aid for therapy with intermittent compression.

• Inpatient treatment phase: Complex physical decongestion therapy with at least one manual lymphatic drainage and compression therapy with short-stretch bandages. If necessary, padding with cotton wool bandages to avoid pressure points. If necessary, additional intermittent pneumatic compression therapy. Nocturnal elevation of the extremity. Physiotherapy.

• Notice! The use of diuretics for lymphedema is viewed with caution, as diuretics are only effective in low-protein oedema. Otherwise, paradoxical effect by accumulation of protein in the interstitium possible!

• The following should always be avoided on the affected extremity: injections, infusions, cold and heat treatment, blood sampling, acupuncture, blood pressure measurement, kneading massage, invasive procedures, spreading of the oedema.

• Anticoagulation (5,000-10,000 IU heparin s.c. 2 times/day).
• If necessary, infection prophylaxis: Intermittent antibiotic therapy (Penicillin G 10 Mega IU for 10 days i.v. every 3 months).

Development of lymphedema according to Földi

• Lympha-mat of the company Bösl Medizintechnik GmbH
• Hydroven from HNE Healthcare.

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