History and Examination

• Family history, as thought to be genetic in approximately 50% of cases.
• Bilateral symmetrical enlargement of the lower and in some cases the upper extremities almost exclusively in women leading to significant disproportion of the waist to hip ratio.
• Onset often at puberty or times of hormonal change (pregnancy, commencing contraceptive pill and rarely the menopause)
• Losing weight does not achieve significant volume reduction in the affected areas (unlike simple obesity), resulting in a disproportionate body shape.

Signs and Symptoms

• "Swelling" of lower limbs from the hips to the ankles. Affected tissues are loose and floppy. Collections of fatty tissue to the medial aspect of both knees.
• Feet and hands are generally spared, creating a 'cuff' appearance at the ankles or wrists.
• Skin tenderness in the limbs and tender to touch.
• Heaviness and pain in the legs.
• Pain in knee joints.
• Bruising easily and spontaneously, often unexplained.
• Skin may be cool to touch.
• Stemmer's sign is negative in early lipoedema.
• Seconadry lymphoedema may develop in established cases. The patient will therefore have a combination of clinical signs of lipoedema and a distal lower limb lymphoedema.

Differential Diagnoses

• Lymphoedema
• Obesity

Obesity

• Purely clinical but need to exclude other co-morbidities.
• Hip to waist ratio greater than 0.85 is often not indicative of lipoedema (unless has been complicated by obesity).

• Skin care – hygiene and moisturising, to prevent injury and infection.
• Exercise – low impact (to assist with weight management and also to stimulate lymphatic drainage).
• Elevation – of legs when sitting, to sleep in a bed (in patients that have developed lymphoedema secondary to their lipoedema
  ).
• Compression stockings - used to support the lipoedematous tissues which may improve symptoms of pain
  (although some patients have difficulty tolerating compression due to tender tissues). Compression hosiery is also used to treat coexistent secondary. lymphoedema. Use of compression hosiery in a patient with early lipoedema may prevent progression to secondary lymphoedema but as yet there is no supportive evidence.
  
• Weight management – Lipoedema is exacerbated by weight gain
  /obesity.Therefore, patients must adopt a healthy diet and be able to maintain and/or reduce their weight.
  

Referral Criteria

The referral criteria can be found on the North Devon Healthcare Trust intranet.

Patients are unlikely to benefit from treatment if they have: a BMI over 40 who are unwilling to see a dietician or follow a Weight Management programme, unwilling to wear compression or a likelihood of non-concordance.

North Devon Lymphoedema service

Referral Instructions

North Devon/North Cornwall – Age 18 years +. By letter or attached referral form, with medical history, medication and the results of any investigations.

Referral Forms

North Devon – By letter or referral form

Patient Information

Lipoedema Ladies

Lipoedema UK

NHS.uk/conditions/lipoedema

Evidence

eLearning: Lipoedema - An Adipose Tissue Disorder

Wounds UK - Best Practice Guidelines: The management of lipoedema

Pathway Group

This guideline has been signed off on behalf of NHS Devon.

Publication date: February 2018