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An elephantine problem

Published:February 24, 2017DOI:https://doi.org/10.1016/j.ejim.2017.02.016

      1. Clinical description

      A 40-year old male belonging to northern India presented to the Emergency Department with multiple wounds on the feet. He had history of long standing swelling of both lower limbs. He had no recent episodes of fever. There was no history of easy fatigability, dyspnoea at rest or with exertion, cardiac illness, jaundice or chronic alcohol intake. There was no history of prolonged immobilization. He was not on any medication and had no previous surgical history. His hemoglobin was 15.3 mg/dl, total leucocyte count was 5400/mm3 and liver and kidney functions were found to be normal. Serum albumin was 4.5 g/dl. Examination revealed grossly swollen bilateral lower limbs associated with dermatosclerosis, papillomatous lesions and hyperkeratosis. There were deep wounds on the dependent parts of the feet. (Fig. 1).
      Fig. 1
      Fig. 1Right (a) and left (b) lower limbs of the patient with characteristic “pachyderm” or “elephantine” skin changes seen in Chronic Filarial Lymphedema and secondarily infected wounds seen as necrotic patches on lateral aspect of left leg.
      What is the diagnosis?

      2. Discussion

      Filariasis is the most common cause of lymphedema worldwide, particularly prevalent in South-East Asia including India and Sub- Saharan Africa [
      • Ndeffo-Mbah Martial L.
      • et al.
      Global elimination of lymphatic filariasis..
      ]. It is a neglected, debilitating disease with clinical manifestations ranging from acute fever, malaise and lymphangitis to incapacitating chronic lymphedema. It is caused by the mosquito-borne nematodes Wuchereria bancrofti and Brugia malayi. These thread-like adult parasites reside in the lymphatic channels or lymph nodes. Lymphedema may progress to grossly disfigured, large limbs or gentials covered by hyperkeratotic papillomatous skin, resembling the elephant skin texture, thus appropriately called “elephantiasis”.
      The principle changes are a result of inflammatory damage to the lymphatic vessel walls, leading to dilated and thick walled lymph channels. Infiltration by inflammatory cells destroys lymph valves causing lymphedema. Chronic lymphedema and stasis results in skin changes of brawny edema and hyperkeratosis occurs. Soon, fissuring of the skin and superadded bacterial infections develop.
      Diagnosis of acute filariasis is made by detection of microfilariae in the blood or other body fluids. Lymphoscintigraphic imaging may be used to demonstrate widespread lymphatic abnormalities. Diethylcarbamazine (DEC) is the drug of choice for treatment of active filariasis, although albendazole has also been used [
      • Ndeffo-Mbah Martial L.
      • et al.
      Global elimination of lymphatic filariasis..
      ]. Treatment of chronic filarial manifestations, on the other hand, emphasizes on hygiene, physiotherapy and prevention of secondary bacterial infections. Although several excisional procedures for elephantiasis have been attempted, the outcomes are less than satisfactory for most.

      Conflict of interest

      No conflicts of interest to declare. No grants taken.

      Reference

        • Ndeffo-Mbah Martial L.
        • et al.
        Global elimination of lymphatic filariasis..
        Lancet Infect Dis. 2016; https://doi.org/10.1016/S1473-3099(16)30544-8