Lymphedema (overview)I89.00

Due to a congenital (primary lymphedema) or acquired (secondary lymphedema) lymphangiopathy, insufficient transport capacity of the lymphatic vascular system, which can no longer or only insufficiently remove the normally occurring lymphatic protein load. In contrast to the "painful blue leg" (phleboedema), painless lymphoedema can be described as a "thick white leg". The edema is relatively coarse.

Based on their etiopathogenesis, lymphedemas are divided into (rare, usually genetic) primary lymphedemas and non-genetic (acquired) secondary lymphedemas. The group of unclassifiable idiopathic lymphedema is included in the genetic lymphedema group.

Primary lymphedema: V.a. 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:

• Lymphedema, Nonne-Milroy type.
• Lymphedema, type Meige (corresponding to lymphedema, type Nonne-Milroy with further associated symptoms)
• Lymphedema-distichiasis syndrome
• Yellow-nail syndrome
• Noonan syndrome.
• Lymphedema and other hereditary disorders (very rare):
  • Aagenaes syndrome
  • Aarskog-Scott syndrome
  • Buschke-Ollendorf syndrome
  • Lymphangiectasia-lymphoedema syndrome (Hennekam syndrome)
  • Hypotrichosis-lymphoedema-teleangiectasia syndrome
  • Kanzaki's disease
  • OLEDAID syndrome.

Unclassified lymphedema:

• Idiopathic lymphedema.

Secondary lymphedema: V.a. in the elderly. Acquired, unilateral or bilateral lymphedema resulting from acquired disorders of lymphatic transport. In the arm, lymphedema following mastectomy is best known.

• Tumors obliterating the lymphatic channels (tumors of the small pelvis (ovarian,uterine, bladder, rectal and prostate carcinomas).
• Chronic infections of the soft tissues (e.g., recurrent erysipelas, herpes simplex, lymphogranuloma inguinale; chromomycosis)
• Direct trauma to the anteriomedial lymphatic vessel bundle (constrictions: medial knee, groin).
• Postoperative condition (e.g. after lymphadenectomies)
• Disturbances of the lymphatic drainage in the wake of radiotherapy
• Parasite infestation (e.g. tropical filarial infestation; see filariasis below)
• Artificial lymphedema (Secrétan syndrome).

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.

Increased circumference and heaviness of extremities, thickening of dermis (underlying lymphostatic fibrosclerosis) and subcutaneous adipose tissue, verruciform epidermal hyperplasia (papillomatosis lymphostatica), postinflammatory hyperpigmentation, onychodystrophy, onychogrypose, disturbance of nail growth. Formation of elephantiasis possible, see Table 1.

Medical history, clinic (local findings, skin fold test/ Stemmer's sign), laboratory.

In case of lymphedema of the lower extremity: Doppler or color duplex to exclude 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, tumors?), computed tomography of the abdomen and upper thoracic aperture; in rare, unclear cases, magnetic resonance imaging (in lymphedema: honeycomb pattern in the subcutis).

For chronic lymphedema:

• Recurrent local arterial and mycotic infections.
• Formation of elastolytic giant cell granulomas
• Combination with immunodeficiencies
• Formation of lymphocysts
• Lymphorrhea
• 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 over 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.

1. Baltaci M et al (2004) Elastolytic granulomas of the legs in familial visceral lymphangiodysplasia with chronic lymphedema. JDDG 2: 766-769
2. Cohen SR et al (2001) Lymphedema: strategies for management. Cancer 92: 980-987
3. Földi E (1985) Pathogenesis of lymphedema. In: Holzmann H, Altmeyer P, Hör G, Hahn K (eds.) Dermatology and Nuclear Medicine. Springer, Berlin Heidelberg New York, S. 395-400
4. Földi M (1996) The current state of the art of drug-based lymphedema treatment. Phlebology 25: 48-52
5. Gaber Y (2004) Secondary lymphoedema of the lower leg as an unusual side-effect of a liquid silicone injection in the hip and buttocks. Dermatology 208: 342-344
6. Hesse G et al (2003) Ultrasound diagnostic techniques in dermatologic angiology and phlebology. dermatologist 54: 614-625
7. Levinson KL et al (2003) Age of onset in hereditary lymphedema. J Pediatr 142: 704-708
8. Muehlberger T et al (2001) Etiology, clinical aspects and therapy of penoscrotal lymphedema. Surgeon 72: 414-418
9. Saaristo A et al (2002) Insights into the molecular pathogenesis and targeted treatment of lymphedema. Ann NY Acad Sci 979: 94-110
10. Vana J et al (2002) Massive localized lymphedema in an extremely obese patient. surgeon 73: 383-386
11. Tiwari A et al (2003) Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surgery 138: 152-161