Lymph fluid is continually drained & filtered through the lymph nodes to move out bacterium , toxins & stagnant cells by lymphatic organisation . The filtered fluid is drained back to the blood stream via lymphatic vessels . Lymphedema is the jut caused when there is damage of the lymphatic system of rules to drain this fluid from the tissue .
Is Lymphedema Hereditary?
Lymphedema can be classified into two types :
Primary Lymphedema : Primary lymphedema is a hereditary condition in which the lymphatic system is anomalous & does not develop properly prior to birth . The symptom of primary lymphedema are not always present at the sentence of birth . In some cases , they can train as lately as in maturity .
Secondary Lymphedema : petty lymphedema condition is mostly do by damage / blockage of lymphatic system or other environmental factors include surgery , radiation , infection or trauma . It is the most common case of lymphedema .
Hereditary Lymphedema Causes, Signs & Symptoms
Hereditary lymphedema is also have a go at it as primary lymphedema . It can be subcategorized into congenital hereditary lymphedema ( hereditary lymphedema type I , Milroy disease ) lymphedema praecox ( hereditary lymphedema case II , Meige disease ) , & lymphedema tarda .
Hereditary lymphedema is inherited as autosomal prevalent trait . Milroy ’s disease is associate with genetic mutation in the FLT4 gene , which encodes for vascular endothelial maturation factor receptor 3 ( VEGFR-3 ) & Meige disease is associated with the chromosomal mutation of ‘ forkhead ’ mob arranging factor ( FOXC2 ) gene .
There is gathering of lymphatic fluid in the hypodermic tissue paper due to deformity , obstruction or underdevelopment of the lymphatic vessels .
The most common associated symptom of hereditary lymphedema is hydrops ( swelling ) of various portion of the physical structure , particularly lower extremities & genitalia due to gathering of protein rich lymph fluid in the interstitial tissues . Apart from swelling of the downcast extremities and/or genitalia , there also might be swelling of the upper extremity , tree trunk & case . In humble extremities , usually , both the extremities are affected & the swelling is most noticeable in the foot & ankle joint , but it is not rare to see swollen thighs . This intumescence is the campaign of uncomfortableness , feeling of niggardness & paresthesia ( tingling sensation ) in the unnatural region . The affected region shows poor lesion healing after a minor psychic trauma ( such as a cut or insect bite ) . The skin of the affected region might become hyperkeratosed with ironic , thick & scaly tegument that gives the appearance of “ woody ” texture .
Milroy ’s disease ( type IA hereditary lymphedema ) is characteristic of congenital excrescence ; however , there have been rare cases in which edema develops later in life . low-toned extremities are the most commonly affected site , but privates might also be affect . Milroy ’s disease might also show signs of up - slanting toenail , papillomatosis , prominent stage veins , urethral abnormalities & hydrocele in scrotum .
Meige disease ( hereditary lymphedema case 2 , lymphedema praecox ) usually develop around puberty or other maturity . to boot to edema of the leg , privates , arm , larynx & face might also be pretend including growth of scandalmongering nail . This is the most common character of hereditary lymphedema accounting for approximately 80 % of the character .
Lymphedema tarda is the patrimonial lymphedema that pass in recent adulthood , usually , after 35 eld of years .
Diagnosis & Treatment
The diagnosis of hereditary lymphedema might be confirmed by thoroughgoing aesculapian history along with clinical evaluation & tomography tests including lymphoscintigraphy , echography , & MRI .
Currently , there is no gene therapy available for transmissible lymphedema as well as no approved medication for the discussion of lymphedema . The treatment of lymphedema is aimed at reducing swelling & prevent infection . perfect decongestive therapy ( CDT ) is used to manage lymphedema by manual lymph drainage along with compression bandaging , skin concern , conditioning use & compression garments . antibiotic can be used for repeated infection or prophylactically & antifungals can be used forAthlete ’s foot .
Various surgical technique have also been adopted to treat hereditary lymphedema include microsurgical anastomosis for rechanneling the lymph fluid into the venous organisation & reduce operations for the removal of excessive fibrotic tissue in terrible cases of lymphedema .
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