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Case 1-2013: Filarial Lymphoedema of Upper and Lower limbs

Talaat Fathy, Ashraf Metwally, Amal A Gouda, Mona Abdelmaksoud
Tropical Medicine Department, Zagazig University Hospitals Case records of Endemic and Tropical Medicine Department, Zagazig University Case presentation:
Differential diagnosis of lymphoedema:
Twenty two-year-old house wife from Sharkeya, Egypt presented to the outpatient clinic of Tropical Medicine Department with fatigue and dissection, irradiation or obstruction by bilateral heaviness and swelling in her lower limbs as well as her left upper limb that started at the dorsum of her feet and progressed gradually to affect both limbs asymmetrically (the left lower limb was swollen more than the right) (Figure 1) up to the knee and then appeared in the left upper limb starting from the hand and gradually progressing to reach level of the elbow. The patient had normal general examination. Examination of the affected limbs revealed normal color, temperature and hair distribution. Staging of lymphoedema: [1, 2]
No visible or dilated veins were noted. The examination revealed no ulceration or any skin lesions in the affected limbs. The oedema was partially pitting with spongy sensation (stage 2).  Stage 0 (latent): The lymphatic vessels have
The circumferences of the affected limbs were measured to observe the response to therapy. The apparent. Transport capacity is still sufficient oedema level was up to knee in both lower limbs and up to elbow in the upper limb (grade 1). The pulsation of dorsalis pedis, posterior tibial,  Stage 1 (spontaneously reversible): Tissue
popliteal, and femoral arteries in both lower is still at the "non-pitting" stage: when limbs were intact as well as radial, ulnar, brachial pressed by the fingertips, the tissue bounces and axillary arteries pulsations in the affected back without any indentation. Usually upon upper limb. There were no palpable inguinal or waking in the morning, the limb or affected axillary lymph nodes. The patient was admitted area is normal or almost normal in size. to Tropical Medicine Department, Zagazig University Hospitals for evaluation of her  Stage 2 (spontaneously irreversible): The
condition. The patient received broad spectrum tissue now has a spongy consistency and is antibiotic and diuretic therapy and was advised considered "pitting": when pressed by the to elevate her lower limbs and wear an elastic fingertips, the affected area indents and holds band over the affected upper limb. The previous measures gave minimal improvement of the swelling. The patient's routine laboratory hardening of the limbs and increasing size.  Stage 3 (lymphostatic elephantiasis): At
performed Doppler evaluation for the venous this stage, the swelling is irreversible and systems in the affected limbs which was normal. usually the limb(s) or affected area is very The nocturnal peripheral blood film was negative large. The tissue is hard (fibrotic) and lymphiscintigraphy for lower limbs revealed undergoing reconstructive surgery, called "debulking". This remains controversial, however, since the risks may outweigh the Fathy et al., Afro-Egypt J Infect Endem Dis 2013; 3(2): 85-88 benefits, and the further damage done to the afflicted with swollen limbs, and 50% of men can suffer from mutilating genital symptoms. [5] associated with high prevalence of filariasis in Egyptian countryside are water, clay soil and Grading for lymphoedema: [1, 2]
different vegetation. Knowing this association According to the extent of involvement in the not only helps mapping of the high prevalence areas but also helps predicting high risk of Grade 1 (mild edema): Lymphedema involves
the distal parts such as a forearm and hand or a Filariasis is usually diagnosed by identifying circumference is less than 4 cm, and other blood film smears, using the "gold standard" known as the finger prick test. The finger prick test draws blood from the capillaries of the finger Grade 2 (moderate edema): Lymphedema
tip; larger veins can be used for blood extraction, involves an entire limb or corresponding but strict windows of the time of day must be observed. Blood must be drawn at appropriate circumference is more than 4 but less than times, which reflect the feeding activities of the 6 cm. Tissue changes, such as pitting, are vector insects. Most cases of elephantiasis are amicrofilaremic in a condition called (occult  Grade 3a (severe edema): Lymphedema is
In conditions where microfilaria can't be seen in present in one limb and its associated trunk blood film, various concentration methods are quadrant. The difference in circumference is applied: membrane filter, Knott's concentration greater than 6 centimeters. Significant skin assays, which detect circulating filarial antigens, are also available for making the diagnosis. The may experience repeated attacks of erysipelas. latter are particularly useful in amicrofilaraemic  Grade 3b (massive edema): The same
cases. Spot tests for antigen are far more symptoms as grade 3a, except two or more sensitive, and allow the test to be done any time,  Grade 4 (gigantic edema): Also known as
Lymph node aspirate and chylus fluid may also elephantiasis, in this stage of lymphedema, the yield microfilariae. Medical imaging, such as CT affected extremities are huge due to almost or MRI, may reveal "filarial dance sign" in complete blockage of the lymph channels. chylus fluid; X-ray tests can show calcified adult Elephantiasis may also affect the head and worms in lymphatics. The DEC provocation test is performed to obtain satisfying numbers of parasites in daytime samples. [6, 7] DISCUSSION
A panoramic look up on this case can help you The most probable diagnosis of this condition is filariasis. Filarial disease is endemic in Egypt in primary congenital type rarely presents late in some villages of Nile Delta governorates where the 3rd decade of life (lymphoedema tarda). [8] it is transmitted by Culex pipiens female. [3] The However, the normal lymph flow excludes this prevalence of filariasis in Egypt is 10-50 case/ insufficiency was excluded by the Doppler study asymptomatic microfilaraemia is higher. [4] the possibility of cancer is excluded by absence Filariasis is considered endemic in tropical and investigations and follow up will be necessary later to exclude the possibility of hidden more than 120 million people infected and one malignancy. The injury of the lymphatic vessels billion people at risk for infection. [4] In isn't supported by history of surgery or fractures in the affected limbs. Podoconisis is a type of lymphoedema that is caused by continuous Fathy et al., Afro-Egypt J Infect Endem Dis 2013; 3(2): 85-88 predisposed individuals that occurs exclusively Spectral and landscape characterization of in the lower limbs. The mycetoma pedis is filarious and non-filarious villages in Egypt. associated with multiple sinus formation on the skin of the affected limb with sulpher granules- like discharge, so it was excluded because of the healthy skin overlaying oedema. Leprosy is excluded because the sensations in the affected parts were preserved and the absence of the 5. Ottesen EA, Hooper PJ, Bradley M, Biswas G The recommended treatment for filariasis is Silva, Nilanthi, PLoS NTDs. 2008(10): 317. 6. Centers for Disease Control and Preventio albendazole is also effective. All of these 7. Hopkins DR: Disease Eradication. N Engl J Med. treatments are microfilaricides; they have no 8. Brorson H, Ohlin K, Olsson G, Svensson B, suggested for treating elephantiasis. [10]Filarial Liposuction Treatment for Lower Extremity parasites have symbiotic bacteria in the genus Lymphedema. Lymphology, 2008, 41: 52–63. to play a major role in both its reproduction and the development of the disease. Clinical trials in completely eliminated microfilaraemia. [11] 10. Hoerauf A, Mand S, Fischer K, Kruppa T, Marfo- REFERENCES
Debrekyei Y, Debrah AY, et al: Doxycycline as a novel strategy against bancroftian filariasis- 1. The WHO Expert Committee on Filariasis: depletion of Wolbachia endosymbionts from Lymphatic filariasis: The disease and its control. Wuchereria bancrofti and stop of microfilaria production. Med Microbiol Immunol (Berl). technical report series 1992,821: 1–71. 2. Tretbar LL, Morgan CL, Lee BB, Blondeau B, 11. Taylor MJ, Makunde WH, McGarry HF, Turner JD, Mand S, Hoerauf A: Macrofilaricidal activity after doxycycline treatment of Wuchereria bancrofti: a double-blind, randomised placebo- controlled trial. Lancet. 2005,365(9477): 2116– Fathy et al., Afro-Egypt J Infect Endem Dis 2013; 3(2): 85-88 Figure 1: Asymmetrical swelling of the lower limbs (more swelling is noticed in the left side). Fathy et al., Afro-Egypt J Infect Endem Dis 2013; 3(2): 85-88

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