Possible presenting signs and symptoms:

Lymphoedema may be unilateral or bilateral depending on the cause/s

• Early stage:
  • Mild and/or intermittent swelling affecting one or more: foot, toes, ankle, lower or upper limb/s, genitals and/or trunk, buttocks, abdomen
• Later stage:
  • Persistent and/or worsening swelling
  • Positive Stemmer’s sign (inability to pinch the base of the second toe due to tissue fibrosis)
  • Shape changes and skin folds
  • Tendency to cellulitis: see cellulitis pathway
  • Skin and tissue changes may include:

• fibrosis (firm, sometimes non-pitting )
• ulceration/fragile skin that breaks easily
• hyperkeratosis (scaly areas)
• papillomatosis (cobblestone skin)
• lymphangiectasia (blister-like protrusion of lymphatics that may leak clear fluid)
• deposition of fat tissue
• lipodermatosclerosis leading to hard indurated tissues
• lymphorrhea (leaking of lymph from the skin)

See Lymphoedema Diagnosis, Treatment and Care  'useful photographs'

Additional information

Stages of lymphoedema defined by the International Society of Lymphology (2013) indicates that lymphoedema is a progressive condition:

Possible causes of lower limb lymphoedema

Cancer-related:

• Primary presentation of a cancer (e.g. prostate, genito-urinary)
• Recurrence of previously treated cancer
• Side effect of cancer surgery and/or irradiation involving lymph nodes and/or local lymphatics, particularly if there has been a wound infection, or repeated surgical incisions
• Advanced, obstructive, metastatic cancer.

Non-cancer-related:

• Primary lymphoedema: may be present at birth or develop at any age, with possible family history and/or genetic element (e.g. Milroy’s disease)
• Previous deep vein thrombosis (post-thrombotic syndrome)
• Chronic venous disease/ulceration: an individual with chronic ulceration may develop persistent oedema when tissue fluid overwhelms the lymphatic system; commonly there is a cycle of ulceration, chronic oedema and cellulitis, further damaging the lymphatics and eventually leading to lymphoedema
• Cellulitis: is both a cause and symptom of lymphoedema
• Non-cancer surgery or traumatic injury such as burn, orthopaedic condition, vascular surgery, bypass graft, congenital strangulation of limb
• Lipoedema leading to lipo-lymphoedema
• Dependent leg (Motor Neurone Disease, Multiple Sclerosis)
• Self-induced injury
• Filariasis infection: where person travelled to India or Africa

Possible worsening or co-morbid factors for lower limb swelling

• Wound infection
• Body Mass Index increase
• Recurrent/metastatic cancer
• Recurrent cellulitis or inflammatory skin condition such as psoriasis or eczema
• Deep vein thrombosis/venous flow abnormalities
• Organ failure (liver/renal/cardiac)
• Medications such as calcium-channel blocking agents, corticosteroids
• Protein-losing disease/hypoalbuminaemia due to any cause
• Poor mobility or function/neuropathic problems/pain
• Person unable to tolerate/apply compression garments.

Investigations to be considered in Primary Care:

• Baseline bloods:
  • Full blood count
  • Urea and electrolytes
  • Liver function tests (LFTs)
  • C-Reactive protein and/or plasma viscosity: to exclude inflammatory conditions that may exacerbate swelling
  • Thyroid function tests (TFTs): to identify thyroid dysfunction that may influence weight and compromise lymphoedema treatment outcome
  • Blood glucose: to screen for diabetes (patients with lymphoedema already have an increased risk of cellulitis)
  • Serum protein: to exclude hypoalbumaemia affecting tissue fluid homeostasis
• Physical examination: to identify signs and symptoms of cardiac failure, chronic liver disease, renal disease and chronic inflammation or infection
• ECG, chest X-ray, abdominal or renal ultrasound scan as guided by above findings
• Doppler ABPI: to exclude arterial disease and identify suitability for compression
• Urine dip for protein and blood: to exclude protein-losing kidney disease
• Mycology nail clippings or skin scrapings: to identify chronic infections of skin/nails.

Exclude: RED FLAG

• Deep vein thrombosis: refer to DVT algorithm
• Cardiac failure
• Recurrent cancer: refer to cancer services
• Musculo-skeletal injury.

Consider referral for secondary care-based investigations or treatment:

• Vascular referral: to exclude treatable vascular disease and identify suitability for compression therapy if ABPI cannot be performed in primary care.
• Orthopaedic, rheumatology or physiotherapy referral: to manage musculo-skeletal problems that affect mobility and exacerbate swelling
• Dermatology referral: to manage skin or nail conditions that may be contributing to swelling or cellulitis.

Tertiary referral for specialist lymphatic investigations and support:

A child or young person with a new or suspected diagnosis of primary lymphoedema may require lymphoscintigraphy and genetic counselling in a specialist centre:

Lymphoedema Service
St George's University Hospital
Blackshaw Road
Tooting
London
SW17 0QT
Tel: 0208 7251784

Differential diagnosis: Lipoedema

• Mainly affects women
• Abnormal distribution of fat tissue
• Limited response to weight reduction
• Usually affects bilateral lower limbs and may also affect arms and buttocks (leading to pear-shape)
• Feet are often not affected
• Skin feels soft and ‘fatty’; there may be tender areas and tendency to bruising
• Stemmer’s sign is not positive unless lipo-lymphoedema has developed when there is an element of lymphatic failure.   

More information is available:

• Talk Lipoedema
• Lipoedema UK Website

Initial self-care support

• Assess extent of swelling, and impact on daily life
• Commence emollients (refer to Highland Formulary):

• use a bland emollient such as Diprobase® cream (500mg pump dispenser) or Epaderm® ointment
• consider Oilatum® as bath additive or Dermol® 200 shower emollient
• if risk of minor skin infections, consider E mulsiderm® as bath additive

• Manage cellulitis/recurrent cellulitis: refer to cellulitis pathway
• Manage skin and tissue changes
• Consider prescribing compression garments: European compression class garments are commonly used for people with lymphoedema. British compression class stockings are only suitable for mild venous oedema
• Avoid routine diuretic use:

Diuretics do not improve lymph drainage and are not indicated for managing lymphoedema. They may reduce swelling and soften the tissues in the short term, but lead to tissue fibrosis. However, spironolactone may be indicated for someone with advanced cancer and intractable swelling.

Advice may be obtained from: nhsh.highland-lymphoedema@nhs.scot And the Lymphoedema Advice Line: 07870 483860

Mild/controlled lymphoedema may include:

• Oedema confined to below knee
• No/minimal toe oedema
• Oedema generally soft and pitting to pressure
• Minimal skin changes- scaly, dry
• Limb well shaped - fits standard compression garment.

Moderate to severe, uncontrolled or complicated swelling may include:

• Oedema that involves the whole leg, thigh, buttocks, abdomen, genitals
• Toe oedema
• Problematic skin changes: hyperkeratosis, cobblestone papillomatosis, ulceration
• Recurrent cellulitis
• Poorly shaped limb that requires a custom-made compression garment

We recommend referral to specialist lymphoedema services if:

• Primary lymphoedema is suspected in a young person or baby
• Lymphoedema is cancer-related: initial assessment by a lymphoedema practitioner is recommended
• There are complex symptoms associated with advanced cancer/end of life.

Referral for specialist treatment:

• A lymphoedema practitioner/clinic
• Specialist nurse: Dermatology, Tissue Viability, Vascular

Lymphoedema Practitioners provide:

• A comprehensive lymphoedema assessment and treatment plan
• Complex compression bandaging using a short-stretch bandaging system, and support to local team on bandaging
• Skilled measurement and fitting of compression garments
• Kinesio-taping: a light hypo-allergenic tape that enhances lymph drainage and is applied to the skin according to individual need.
• Manual Lymphatic Drainage (MLD): a specialist type of massage mainly used for those with breast, trunk or genital swelling that enhances lymph drainage.
• Teaching self-massage, an adapted form of MLD to patients and carers
• Specific exercise and lifestyle advice
• Self-management courses.

Management may include:

• A comprehensive lymphoedema assessment and treatment plan
• Compression bandaging using a short-stretch bandaging system, with or without manual lymph drainage (MLD) massage. Bandages may be changed 2-3 times per week, and often include toe and full leg bandage, to reduce swelling, improve shape and skin conditions.
• Manual Lymphatic Drainage (MLD) massage: a gentle skin massage that enhances the work of the lymphatic system, redirecting fluid towards healthy lymphatics, without increasing capillary filtration. It is useful for individuals with trunk swelling.
• Measurement and fitting of standard and custom-made compression garments (European compression class and flat-knit garments are commonly used)
• Kinesio-taping: a specialist hypo-allergenic tape that enhances lymph drainage; particularly useful for trunk and breast oedema
• Specific exercise and lifestyle advice
• Support to patient and family.

Manage and review in primary care by a suitably trained practitioner such as:

• A lymphoedema practitioner/clinic
• A local link practitioner
• A nurse specialist in Dermatology, Tissue Viability or Vascular
• A hospital or community nurse with appropriate training.

Management may include:

• Care of skin problems
• Fitting of compression garments: patients may require a regular supply (every 3-6 month) of repeat garments, with appropriate follow up care
• Support with self-care including advice on skin care, exercise and weight management
• Referral to a self-management course, exercise group or support group
• Support to patient and family.