Lipedema R60.9

Chronic and progressive disease that occurs almost exclusively in women and is characterized by a fat distribution disorder with marked disproportion between trunk and extremities. It is caused by a circumscribed, symmetrically localised subcutaneous fat tissue proliferation of the lower and/or upper extremities. In addition, there are oedemas, which are intensified by orthostasis, and a tendency to hematoma after minor traumas. Increased pain under pressure is also characteristic; mostly there is spontaneous pain.

Lipedema is classified according to localization and morphology:

a) Classification of lipedema according to localization (mod. according to Herpertz 2014):

• Legs: thigh type, lower leg type, whole leg type
• Arms: upper arm type, forearm type, whole arm type

b) Classification of lipedema according to morphology:

• Stage 1: Smooth skin surface with evenly thickened, homogeneously imposing subcutis
• Stage 2: Uneven, predominantly wavy skin surface, nodular structures in the thickened subcutaneous area
• Stage 3: Pronounced circumferential propagation with overhanging tissue parts (dewlap)

Progression to lipedema with secondary lymphedema - partly depending on comorbidities (e.g. obesity, inactivity) - possible in every stage. Accompanying obesity can worsen the course and symptoms of lipedema.

There is no reliable data on epidemiology from large studies. Existing small studies show a high variance with prevalences between 0.1% and 18% depending on the collective studied (general population, selected patients). Overall, a high number of unreported cases and false diagnoses are likely in view of the widespread uncertainty regarding the diagnosis.

The etiology is still unknown. The exact pathomechanisms as well as the specific role of hormones and their receptors are also not understood. A specific biomarker is missing. In up to 60% of cases a genetic component with familial accumulation of lipedema has been described. For example, an autosomal dominant inheritance pattern with incomplete penetrance has been described in six families with lipedema over three generations.

The described adipose tissue proliferation is the result of hypertrophy and hyperplasia of the fat cells. In addition, there are changes in the connective tissue, capillary permeability disorders with a consecutive tendency to hematoma.

In the advanced stage, secondary changes such as sclerosis and papillomatosis due to fibroblast proliferation in the dermis become apparent. Increasing fibrosis of the subcutaneous fatty tissue leads to mechanical insufficiency.

Almost exclusively in women. Typically begins in a phase of hormonal changes such as puberty, pregnancy or climacteric. In men, lipedema-like changes are described in hormonally effective therapies, pronounced hormonal disorders (e.g. hypogonadism) or in liver cirrhosis.

Symmetrically localized at the lower (more frequent) and upper (less frequent) extremities.

Always symmetrical, disproportionate fat increase on arms and legs.

Typical is a "calibre jump" to the adjacent healthy region ("muff", "Turkish pants phenomenon", "collar formation").

Haematomas of different age and colouring

Hands and feet always remain free.

Stemmer sign negative.

Accompanying: pain, oedema, sensation of heaviness and tension in the affected extremities.

Often significant reduction in quality of life.

The stages are not necessarily the same as the extent of clinical symptoms (pain).

- In the later course, often circumscribed bulge formations (dewlaps) are also found, which are mainly located on the inner sides of the thighs and knees, more rarely also in the ankle area.

- Secondary effects due to chafing (chronic irritative dermatitis), occlusion (macerations and consecutive infections) and gait disorders (axial malposition of the legs, especially valgus gonarthrosis).

- In rare cases (about 50-60 cases are known) lipedema is found in combination with analbuminemia.

The histological changes in lipedema are not pathognomonic. In addition to increased and partly hypertrophic fat cells, the interstitium shows a high content of capillary blood vessels; perivascularly, macrophages, fibroblasts, mast cells and isolated fat tissue necroses are found. In the late stage of the disease the fibrotic part increases.

Immunohistological investigations showed degenerative and regenerative changes in the fatty tissue, characterized by crown-like structures of necrotic adipocytes surrounded by infiltrating CD68+ macrophages and proliferations of fat-associated stem/progenitor and connective tissue cells (Ki67- and CD34-positive). These findings support the thesis of an increased adipogenesis in the tissue of the lipedema.

- Diagnosis is typically made solely on the basis of medical history and clinic.

- High resolution sonography: differentiation between lymphedema, lipedema and phlebedema. Characteristic are homogeneous widening of the subcutis, uniform echogenicity ("snow flurry"), presentation of echoric septums. Useful supplement to differential diagnosis, but no specific characteristics.

- Indirect lymphography: Differentiation of lipedema, lipolymphedema and lymphedema on the basis of different figures of injection depots and lymph drainage via the lymph collectors.

- Functional lymph scintigraphy (lymph drainage scintigraphy): Initially increased lymph transport; in advanced stages, evidence of slowed lymph transport.

- Further diagnosis only useful to exclude other causes of oedema.

- Use of BMI, hip-waist index or size-waist index to differentiate between obesity, especially recommended for monitoring progress. It is also useful to measure the circumference and volume of the extremities during the course of the procedure to objectively assess the findings.

Obesity: symmetrical and uniform, soft fatness of the whole body surface; percentage of fat mass exceeds 30% in women and 20% in men.

Obesity with fibromyalgia: symmetrical and uniform soft adiposity of the whole body surface with pain.

Lip-lymphoedema (Lipolymphoedema) - Remark: To what extent both clinical pictures differ, remains reserved for further investigation results.

Lipomatosis dolorosa: painful lipomas

Lipomatosis, benign, symmetric: familial occurrence; symmetric soft adiposis of the extremities

Phleboedema: oedema in chronic venous insufficiency - CVI; always skin sign of CVI

Pretibial myxoedema: firm doughy oedema of the lower legs

Cardiac edema: lower leg and foot, changeable, increased during the day

In orthostasis, easily indentable edema of the tibia and ankle region develops. These are signs of additional lymphedema (lipo-lymphedema). With increasing fatty degeneration of the legs, the legs become increasingly sensitive to pressure (especially in the medial knee region, see painful lipo-lymphedema syndrome below).

Minor traumas lead to haematomas. In about 30% of the cases psychological disorders occur.

Causal therapy and healing are not yet possible.

The therapy pursues two goals:

1. The elimination or improvement of findings and complaints (especially pain, edema and disproportion).
2. The prevention of complications. As the findings become more pronounced and the leg volumes, in particular, increase, the risk of dermatological (e.g. macerations, infections), lymphatic (e.g. erysipelas, lymphoedema) and orthopaedic complications (gait disorders, axial malpositions) increases.

The symptomatically effective measures are carried out in a stage-specific and individualized manner.

• Complex physical decongestion therapy (KPE) with: manual lymph drainage, compression therapy (usually flat knit class II or III, split fittings more suitable for everyday use; in the case of initial lipedema, circular knit with high material strength, class II),
• Exercise (especially suitable for water sports)
• Optimized main care
• if necessary, also apparative intermittent compression therapy (e.g. lympha-mat or hydroven).

KPE is useful in cases of pronounced edema and pain.

Surgical therapy:

• Liposuction under tumescent anaesthesia with vibrating cannulas (results remain to be seen, but the procedure is offered in specialised centres; often the patient's psychological stress is considerably reduced by the rapid success of the therapy). The long-term success of these therapeutic measures should be proven in larger studies. Surgeons experienced in liposuction recommend a critical indication with a body weight > 120kg or a BMI > 32 kg/m2 . Morbid obesity accompanying lipoedema should be treated therapeutically before liposuction. Ultimately, the indication and the performance of the liposuction are at the discretion of the surgeon.
• In the case of pronounced dewlap formation, a plastic-surgical reduction may be useful.

Nutrition: although there is no lipedema-specific diet, an isoglycaemic diet with avoidance of high blood sugar and insulin levels and sufficient breaks between meals has proven to be effective. Weight reduction should not be at the expense of muscle mass, but of fat mass. However, lipedema-specific configuration does not disappear as a result. According to the S3 guidelines of the German Obesity Society, indications for weight reduction are a BMI of 30 kg/m2 or a disease that can be aggravated by overweight, as is the case with lipedema.

Psychotherapy: among the patients often different eating disorders. In these cases, a change in diet should be made with psychological support.

Drug therapy: no diuretics for lip and/or lymphedema.

Chronic progressive course, but very different from individual to individual. Simultaneously existing obesity can worsen the course considerably. To monitor progress, it is recommended to document further parameters such as weight, body mass index (BMI), orphan hip ratio, circumference and volume measurements of the extremities and the daily activity idex (Reich-Schupke S et al. 2017).

It makes sense to reduce any existing overweight. Weight loss cures, special diets or diuretic therapy are not indicated.

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