Lymphedema is a pathological condition caused by the accumulation of body fluid in the superficial soft tissues due to obstruction of lymphatic flow or retention of lymphatic fluid, followed by fibrous hyperplasia, fat sclerosis, fascial thickening and thickening of the entire affected limb. The skin is thickened, rough and hard like elephant skin, so it is called “elephant skin disease”. In Chinese medicine, it is called “canker sore” disease.
1. Etiology and pathology.
There are many causes of lymphedema and many ways to classify it, and they are classified according to etiology as follows
(1) Early-onset lymphedema: This disease occurs mostly in adolescent females, and most of them are within 25 years of age. The etiology of this disease is not clear and may be related to the physiology of the reproductive system. The rapid development of reproductive organs during adolescence increases the load on the pelvic lymphatic system, which subsequently leads to lymphatic insufficiency of the lower extremities, and may also cause the same result through infection of the pelvic lymphatic vessels and lymph nodes.
(2) Congenital lymphedema: congenital abnormal development of lymphatic vessels, which cannot carry lymphatic fluid causing swelling of the lower limbs.
(3) Malignant tumor cell obstructive lymphedema: malignant tumors are commonly seen in cases of cancer of the breast, uterus, prostate, bladder, testes and skin or bones; others such as Hodgkin’s disease and lymphangioleiomyosarcoma can also be seen occasionally, and all these malignant tumors can obstruct the lymphatic vessels. Regional lymph node metastasis not only blocks lymphatic circulation, but may also contribute to retrograde tumor cell metastasis. Retrograde metastasis of uterine body cancer cells to heel skin, breast cancer cells to upper limb skin, and bladder cancer cells to penis have been reported. Therefore, for some patients with lymphedema, we should try our best to find the cause of lymphedema.
(4) Post-surgical lymphedema: It usually occurs after malignant lymph node dissection or tuberculous lymph node dissection, which affects the lymphatic return of local tissues. Some degree of swelling of the upper extremities usually begins after resumption of postoperative activities, but there are cases in which symptoms begin weeks, months, or even years after surgery.
(5) Lymphedema after radiation therapy: Radium ingot or deep X-ray therapy is an important factor causing tissue fibrosis. As a result of repeated radiation therapy, tissue fibrosis and fibrous tissue proliferation are induced, affecting lymphatic reflux and eventually forming lymphedema.
(6) Injury lymphedema: Any injury, such as injury to soft tissue, fracture, bleeding, over-tight bandage, plaster fixation of dangling position and secondary infection factors, can inflame the lymphatic vessels and cause lymphatic inflammatory edema, and if it lasts for a long time, it causes lymphatic vessel obstruction and tissue fibrosis, resulting in permanent swelling of the limb and affecting the function of the limb.
(7) Filariasis lymphoedema: In areas where filariasis is endemic, lymphoedema caused by filariasis is often a late complication of filariasis. Once it is advanced, the filarial worms or worms have died and microfilariae are not easily detected.
(8) Other inflammatory lymphedema: tinea pedis, dermatitis, injury, gynecological disorders, and varicose veins of the lower extremities can cause lymphedema.
The pathology of lymphedema suggests that lymphangitis, regardless of the cause, causes lymphatic vessels to become obstructed. Once the lymphatic vessels are blocked, the lymphatic fluid reflux in the distal end and its side branches and distal soft tissues belonging to them is obstructed, causing a large accumulation of lymphatic fluid, which forms lymphedema, and over time, causes the chronic fibrous connective tissue within and under the skin to proliferate and hypertrophy, resulting in persistent enlargement of the limb.
In the early stage of the disease, the lymphatic vessels are acutely blocked by its inflammation, causing obstruction of lymphatic fluid return and producing edema in the tissue spaces. The high content of lymphatic fluid proteins in the resting state leads to coagulation of exudate. The conditions are provided for the proliferation of fibroblasts. Over time, a large amount of fibrous tissue is produced in the intradermal and subcutaneous tissues, and the damage to the lymphatic vessels is exacerbated. Clinically common: the skin is gradually hypertrophied, its surface is keratinized and rough, hard as elephant skin, and even warty growths, cracks or ulcers appear. The subcutaneous area is extremely thickened by degeneration of fatty tissue and proliferation of fibrous connective tissue, forming a typical “elephantiasis”.
According to Chinese medicine, stagnation of the meridians, blockage of the veins and channels, difficulty in the movement of qi and blood, resulting in swelling of the limbs, is caused by dampness and heat, often due to poisonous qi entering the meridians or insect bites, or congenital deficiency.
2.Clinical manifestations
In early stage lymphedema, there is no significant change in the structure and form of the skin, the symptoms are sometimes mild and sometimes heavy, and the swelling of the limbs often changes due to the change of position. In late stage lymphedema, it is very easy to diagnose, and the affected limbs show symptoms of extreme thickening and typical “elephant skin”-like skin characteristics.
(1) Filariasis lymphedema: In the early stage of the disease, due to the accumulation of edematous tissues for a long time, the local resistance to disease is weakened and secondary infection by Streptococcus haemolyticus is easily caused. As a result of the infection, the inflammatory exudate can be deposited in the intradermal and subcutaneous tissues due to its extremely high protein content, forming a large amount of fibrous connective tissue, as well as damaging and obstructing more lymphatic vessels. Further damage to the lymphatic vessels makes lymphatic fluid retention more severe, and with localized focal infections such as tinea pedis, secondary inflammatory DDangium-like recurrences become more frequent. For this reason, the two factors, lymphatic obstruction and infection, become causal to each other, forming a vicious circle.
In advanced cases, the tissue becomes extremely fibrotic, and all elastic fibers break and lose their function, resulting in a complete loss of the original elasticity of the skin and subcutaneous tissues and the formation of a typical elephantiasis. Because the disease is mostly seen in the lower extremities, it is often called “elephantiasis”. In fact, the upper limbs, penis, scrotum, labia majora and other parts of the body can occur.
In China, lymphedema in the scrotum occurs mainly in the lower extremities, followed by the scrotum in areas where filariasis is endemic. It is less common for the penis and scrotum to develop at the same time, and even less common for it to occur in the penis alone. In advanced cases, the scrotum is enlarged to more than 10 kg, usually around 2-4 kg.
(2) Symptoms of Dantoid-like lymphoma: a typical attack, usually without prodromal symptoms. During the attack, there are severe chills, nausea, vomiting, and a rise in body temperature to between 38-41 degrees C. At the same time or a little later, a small area of redness and swelling appears in one of the affected limbs, which gradually increases in extent as well as spreading to the entire affected limb. The inguinal lymph nodes are enlarged and painful. Chills may occur several times with a gap of 0.5 to 1 hour. High fever may last from a few hours to 2-3 days. During high fever, the patient feels peripheral discomfort, which may remain for 1-2 days after the fever subsides.
3. Physical signs.
Mainly swelling of the limbs, but the signs vary among different etiological types, as follows.
(1) Early-onset lymphedema signs: swelling of the ankle joint first, gradually progressing to the entire limb, but complications of cellulitis and lymphangitis are not uncommon. The disease progresses progressively, not only with depressed edema in the thick limb but also with rough and hardened skin, probably as a result of fibrous tissue hyperplasia. These symptoms are aggravated during activity and reduced after rest.
(2) Congenital lymphedema: there is limited or diffuse enlargement of one limb at birth, without pain, ulceration, or infection, and the patient’s general condition is good.
(3) Lymphedema caused by filariasis: The lymphatic vessels of the lower limbs are narrowed, occluded and destroyed due to filarial obstruction, so that the lymphatic flow back to the skin and subcutaneous tissues of the distal limbs to which they belong is blocked, and the amount of lymphatic fluid in the tissues is thus abnormally increased, at which time the calves are uniformly thickened, especially in the middle and lower 1/3. At the beginning, the skin is smooth and soft, and indentations may appear after finger pressure. After elevating the affected limb or resting in bed, the edema can obviously subside. At this time, it is still a lymphedema type. Further development of this disease is “elephant skin leg”. That is, the intracutaneous and subcutaneous tissues begin to show chronic inflammatory fibrous tissue hyperplasia and hypertrophy.
(4) Dantoid-like lymphedema: The disease is characterized by recurrent episodes of acute cellulitis and acute lymphangitis. The superficial tubular lymphatic vessels show a red line, hard and painful, leading from the primary infection to the adjacent nodes, which is called “red silk furuncle” in Chinese medicine. Lymphangitis in the lower extremities tends to the N-fossa and groin, and in the upper extremities to the supraspinatus, axilla, and pectoralis major. Deep lymphangitis can cause swelling and pain in the entire limb. It is accompanied by systemic symptoms. These symptoms are repeated with each attack, with smaller and smaller intervals between attacks. The edema that remains from the first attack is often less severe, but increases with each attack without exception. Generally, the number of attacks becomes more frequent and the local condition worsens, eventually becoming a typical “elephant skin leg”.
4. Ancillary tests.
Blood tests show an increased white blood cell count, and in very severe cases, such as bacteremia and sepsis, bacteria can be cultured in the blood. In early cases, microfilariae can be detected in the blood. In advanced cases, the filarial worms are mostly dead and the microfilariae disappear from the blood. Lymphangiography can clarify whether the lymphatic system is abnormal and whether the lymphatic vessels are obstructed, and can provide technical support for lymphatic-venous anastomosis.
5.Treatment
The aims of treatment for lymphedema are
①To remove the stagnant lymphatic fluid;
②Prevent the regeneration of lymphatic fluid;
(3) Removal of irrecoverable fibrosclerotic tissue.
Treatment methods for lymphedema
①Shunt surgery for limited lymphatic obstruction;
②Baking therapy;
③Excision of irrecoverable fibrosclerotic tissue and trapped lymphatic gap;
④Chinese herbal medicine to clear the heat, water and swelling.
(1) Treatment of acute lymphedema: inflammatory, injury or allergic lymphedema, in the acute stage, should accelerate lymphatic reflux and control infection.
Antibiotic therapy: penicillin 800,000u 4 times daily intramuscular injection; streptomycin 0.5g 2 times daily intramuscular injection; or gentamicin 80,000u 2 times daily intramuscular injection; or Pioneer 4 0.5g 3 times daily orally, as appropriate.
Postural drainage: For upper limb edema, the wrist joint can be suspended on a brace, while sedative or anesthetic drugs can be dispensed to give the patient good rest throughout the body, but care must be taken to protect the wrist joint from ischemia due to excessive restraint, and during treatment, the affected limb can be lowered at intervals for a short period of activity and then suspended again. For lower limb edema, the foot of the bed can be elevated about 30 cm using a chair or wooden pillow. It is not advisable to put pillows on the N, as this is not conducive to venous and lymphatic reflux.
Pressure bandage: pressure bandage helps to stop lymphatic retention and force its function of reflux in the direction of the heart, according to the specific situation, you can use elastic bandage, elastic stocking, glycerin gelatin “boot”, ordinary bandage, elephant skin bandage or other semi-sclerotic bandage products.
Rational selection of diuretics: in the acute edema period and in preparation for surgery, furanilic acid (tachyphylaxis) can be given at 20-40 mg orally once a day or every other day; mercurius, 1-2 mL intramuscularly every time, once every 4-5 days; dihydrocortisone, etc., at 50 mg orally three times a day. The latter two have more significant effects. It should be noted that excessive application of dehydration therapy can compromise the patient’s coagulation function.
Control of salt intake: In the acute edema period, the intake of sodium chloride should be limited to 1-2 grams per day, and drinking water should not be restricted.
(2) Treatment of chronic lymphedema: The treatment of chronic lymphedema is divided into surgical and non-surgical therapies, as follows.
Surgical treatment methods: Depending on the condition, partial excision of lesion, excision of lesion and flap grafting, lymphovascular-venous anastomosis, lymphovascular-venous end-lateral anastomosis, lymphovascular-venous end-end anastomosis and other methods can be used.
Baking and tying therapy: Baking and tying therapy is an effective method in Chinese medicine for treating leg swelling, which uses continuous radiant heat to cause the skin blood vessels of the affected limb to dilate, and a large amount of, local tissue interstitial fluid is returned to the blood, while lymphatic circulation is also improved. Anyone with early lesions of elephantiasis, such as lymphedema, or late lesions of elephantiasis, but without serious symptoms on the skin of the limb, is an indication for baking and tying therapy. For patients who have undergone surgical excision of skin implants, the implantation site of the limb often cannot tolerate high temperature and is prone to rupture, so it is a contraindication to baking therapy. At present, there are two kinds of baking therapy: electric radiation heat therapy and oven method, and electric radiation heat therapy is commonly used at present.
(3) Chinese medicine treatment.
Damp-heat type: during the attack period, the limbs are red, swollen, hot and painful, with generalized hyperthermia and chills; during the remission period, the skin is smooth and soft, the early stage of limb swelling changes with the change of position, and the late stage of swelling does not subside; the tongue coating is thin and white, and the pulse is sunken and thin; for acute lymphedema or chronic lymphangitis acute attack; treatment is appropriate to clear heat and detoxify dampness, and the formula is as follows: honeysuckle, forsythia, cyperus, nux vomica, atractylodes, antibiotics, papaya, poria, Chuanxiong, red flower.
Stasis type: swollen limbs with toughness, obvious after walking, obvious after standing for a long time, light in the morning and heavy in the evening, depressed skin swelling, depressed skin swelling, late skin roughness like orange peel, forming “elephant skin legs”, red tongue, white fur, stringent and astringent pulse; chronic lymphedema stage, treatment should be based on activating blood circulation, eliminating stasis and relieving dampness. The whole formula of Chinese medicine is as follows: Andrographis paniculata, Soapberry, Trigonella, Curcuma longa, Chuanxiong, Safflower, Calamus, Clove, Patchouli, Poria, Papaya, Fangji.