Extra on the Pathophysiology of Lymphedema and on Lymphedema Clinics

First – The function of a lymphatic system:

The primary capabilities of the lymphatic system are the elimination of proteins of excess molecular weight and immunological position (an infection, cancer).

In case of lymphedema, the excessive molecular weight proteins stay in the interstitial fluid, causing a rise in interstitial oncotic pressure, causing edema.

The absence of substitution (the venous system is unable to take away these proteins) explains the persistence of edema (attracted by proteins), even after diuretic therapy (which causes salt depletion with out action on proteins).

Second – implications of lymphedema:

This high protein promotes fibrosis and pores and skin infections.

The presence of proteins and degradation merchandise of collagen stimulates the exercise of fibroblasts answerable for fibrosis.

In lymphedema, there's a hyperplastic fibrosis however no ulceration, in contrast to venous insufficiency.

Bacterial dermohypodermitis or streptococcal lymphangitis complicating lymphedema frequently.

In lymphedema, the pores and skin modifications are primarily dermal (improve in thickness, water retention) but in addition hypodermic (the fats lobules of the subcutis are larger).

Primary lymphedema of the child are often ensuing from lymphatic hypoplasia roughly intensively.

Networks are delaying substitution, in some types, medical manifestations, which will occur through an episode of genital life (puberty, being pregnant), trauma, surgery or irradiation.

Studies of the superior lymphatic network in microlymphographie show a dilated lymphatic system (primary lymphaticema occurs after puberty) or either either full aplasia of superficial lymphatic (congenital lymphaticema type I) or superficial lymphatic ectasia (congenital lymphaticema sort II).

Scientific lymphedema:

Lymphedema will be very early, from the neonatal period or at puberty, or later after 35 years.

The diagnosis is almost always straightforward, including within the newborn and infant (Chubby ordinary look at this age).

Secondary lymphoma is more proximal lymphatic congestion occurring near the blockage.

Upper limb, it's the classic "huge arms" after proximal to early radiosurgical treatment of breast cancer.

The first lymphedema usually start by aggravation of the extremities.

Decrease limit, the event is critical edema of upper foot, which does "non-pitting" within the early forms, this is not constant.

The toes are pudgy, with transverse folds marked, especially at the base.

Stemmer's sign is taken into account pathognomonic of lymphedema of the lower extremity: is thickening of the pores and skin fold, highlighted by pinching the top of the second toe.

Subsequently, lymphedema clears bony ankles (filling areas retromalleolar), giving a facet of leg "publish".

It results in skin fibrosis with fibrous papules, vegetation and deep transverse folds. Other displays are possible.

Lymphedema may be suspended as much as the upper thigh.

It could additionally attain the genitals, face, or be generalized.

In main lymphedema of the limbs, just one will be reached, or each decrease limbs, both higher limbs, higher limbs and decrease limb on the identical side or opposite side.

Lymphedema of the lower limbs could reveal or accompany a loss of enteropathy by malformation of the lymphatic system tract (chyloed? Me, Waldmann syndrome or intestinal lymphangiectasia, lymphangiomatosis …) or an abnormality of the thoracic duct.

Kaposi's sarcoma may be preceded or accompanied by lymphoedema.

In instances of persistent venous insufficiency, lymphatic system abnormalities are observed.

The lymphatic insufficiency, initially dynamic, may over time turn into mechanical, alteration of lymphatic capillaries attributable to venous stasis.

This could explain some scientific abnormalities of submit-thrombotic syndrome and untreated older.

This could additionally explain the pathophysiology fibrosis and certain scientific abnormalities seen in persistent edema, whatever their trigger (anasarca, "elephantitis" Tropical …).