Uncommon manifestations of filariasis.
Filariasis is widespread in India and occurs in all the states except in Punjab (early traces) and Himachal Pradesh. The population at risk has been reckoned as 122 million. The infection is of very long standing since elephantiasis has been reported in India from very early times by famous Hindu physicians like Susruta and Madhavakara (Quoted by Hawking and Denham and Hawking). The common clinical manifestations have been reported in an earlier communication from our institute,,  and have been noticed by others also. However, uncommon pathological lesions may also be caused by three developmental stages of Wuchereria bancrofti.
Presented herein is our experience with 20 cases who had uncommon manifestations of filariasis seen at the Mahatma Gandhi Institute of Medical Sciences, Sevagram, during 1970 to 1982.
In all these cases, diagnosis of filariasis was established by demonstrating microfilariae in the night blood smear. A total of 187 cases had the manifestations of filariasis. Of these, 167 had the common clinical manifestations; however, only 20 had uncommon manifestations. These were abscesses on extremities and breast in 10 cases, hematuria in 3 cases, lymph serotum in 3 cases, and granulomatous lesions and massive localised elephantiasis in two cases each.
Of the 20 cases, 4 were females while the rest were males (ratio 1:4). The youngest individual was 18 years, and the oldest was 48 years. Seventy per cent of these belonged to the third decade. The investigative procedures undertaken in our cases are shown in [Table - 1]
There were 7 cases of abscess on extremities which started as lymphangitis and were associated with high fever, chills and rigors. In all of them either the inguinal or axillary lymph nodes were acutely inflammed. The pus culture was sterile. Similarly, the breast abscesses also had the classical features like swelling, tenderness, oedema, in addition to pyogenic axillary adenitis. Incision revealed pus-like fluid in a closed sac. Miscroscopic examination showed motile microfilaria. This pus was reported as sterile on culture examination. Hematuria was associated with filarial fever. This was profuse, total but intermittent. The duration varied from 3 months to 2 years. Cystoscopic findings included granulomatous lesions around ureteric orifices and in the area of trigone associated with marked congestions and ulcerations of mucosa. All the freshly voided samples of urine revealed sluggishly motile microfilariae.
Two of our cases had granulomatous lesions. In the first case this was detected in a breast lump which was clinically diagnosed as antibioma as it started like a pyogenic condition; however, subsequently it became a definitive lump under antibiotic therapy. The other case presented with episodic, intermittent right groin pain in an undescended testis. He complained of painful swelling of this testis associated with typical filarial fever, which responded to diethylcarbamazine therapy.
In both these cases the excised specimen revealed multiple nodules varying from 3 to 7 mm in size on cut section. The histology in these cases came out as a chronic granulomatous lesion.
Two of our cases had massive localised elephantiasis confined to the lower part of the leg and foot only. The duration was also considerably short varying between 2 and 3 months. The skin was enormously thickened, and of firm consistency with multiple rugosities. There was no pitting on pressure owing to overgrowth of the connective tissue. This swelling was confined only to the leg, which acquired the dimensions similar to the thigh. In both these cases the dorsum of. the foot was swollen and tense. The patient experienced marked heavyness during walking.
Cases of lymph scrotum presented to us with multiple vesicle formation on the scrotum varying from 1/2 to 11/2 cm in diameter [ Fig. 1]. Rupture of these due to friction, led to profuse exudation of lymph varying from 2 to 1 litre in quantity. Often during the attacks of fever, the scrotum kept on dribbling the lymph. The patient often complained of wettening of undergarments occasionally during the whole day and night which spontaneously stopped by itself or with diethylcarbamazine therapy. Microscopic examination of the lymph often revealed swarming microfilaria.
The various surgical procedures adopted by us in them were; incision and drainage for abscesses, excision of the breast lump, orchiectomy and hernia repair for undescended but chronically inflammed testis, Thompson's wide excision for elephantiasis, partial scrotectomy for lymph scrotum. Pre-operatively, all these cases were treated with diethylcarbamazine forte injections for 5 days followed by tablets three times a day for three weeks.
While the common manifestations of filariasis include inflammatory reactions of lymphatic systems and/or sequele of their obstructions and fibrosis or rupture leading to miscellaneous effects, uncommon pathological manifestations are increasingly reported in the literature. It is to be remembered that pathological lesions are caused by three developmental stages of the parasites in the human host namely developing larvae, adult worms and the microfilariae. Developing larvae and adult worms bring about changes in the adjacent tissues producing characteristic lesions of classical filariasis. Microfilariae are invariably found in the blood but are reported to cause seldom lesions.
The swarming population of microfilariae in abscesses presenting as pyogenic conditions enlightens the fact that these lesions need not be confined to the lymphatic system but can be associated with other organs like liver, lung, spleen and subcutaneous tissues. The toxins liberated by these microfilariae provoke immence cellular inflammatory reaction resulting into an abscess. These have typical features of inflammation, localization redness and tenderness. The exudative tissue fluid and chyle show different proportions in such localization. In fact, the abscess is an exudative reaction of the tissues, the chyle and swarming microfilaria, often with eosinophilic cells. Thus clinically, the acute inflammatory reaction has chyle like exudative fluid which was proved sterile in our cases. In all our cases, therefore, what we thought as pus was infact the exudative fluid in term and acute local reaction simulating abscess.
Different individuals and tissues show different hypersensitivity reactions to the filaria antigens. It is now believed that any hypersensitive person to a specific filaria antigen can develop eosinophilic tissue reaction especially when the adults continue to produce microfilariae for a long time. Subrahmanyam et al utilized these antigenic properties in performing skin tests in diagnosing chronic filariasis. The results are encouraging and would be published soon.
Our cases of haematuria had definitive findings like ulceration, congestion and granulomatous reaction; however, Nigam et al observed only essential hematuria in filariasis. Bhaskaran et al from India reported twelve cases of filarial splenic granulomata caused by Wuchereria bancrofti, and eosinophilic lesions with abscess formation by microfilariae in lymph nodes. Further, they noted microfilariae in various cut sections of lesions with tissue reactions in the heart, lung, kidney, brain and breast tissue. We noted granulomatous reactions in the breast and testis. The pathology of elephantiasis is well recognised.
The term lymph scrotum is applied to a condition of dilatation, tortuosity and excessive rugosity of cutaneous lymphatics of the scrotum.
Often, these lymphatics appear like vesicles which vary in size from a few mm to cm or more. The delicate lining is subject to pressure from time to time from both of the external and internal in nature. Local friction may cause rupture and lymphorrhagia, lasting for a few days; however, during acute filarial fever lymphatic rupture can cause profuse and persistent lymphorrhagia.
We believe that lymph scrotum is a stage in the development of elephantiasis of the scrotum which is uncommonly seen in practice. Repeated attacks of friction and infection not only lead to lymphorrhagia but also accumulation of lymph in the subcutaneous lax tissues of the scrotum. This provokes extensive fibrosis and thickening of the scrotal skin which is well documented as elephantiasis. In females the skin of vulva may become excessively thickened and hard. Such localized swelling which is continuous with healthy tissue has been designated as `elephantoid tumor' by us. These cases had definative localized thickened areas around the vulva [ Fig 2]; in the lower extremities however, other filarial stigmas in different forms were not observed in them.
Search of literature revealed uncommon but interesting clinical manifestations of filariasis. Nagaratnam et al reported eight cases of benign obstruction of subclavian and axillary veins due to filariasis. These patients had classical swelling of the arm with dilated superficial collaterals. Axillary phlebography revealed typical obstruction. Other parasitic infections namely Loa-Loa and Depetalonema perstans may occasionally develop some neurological complication. Bada presented a case with cerebellopontine angle tumour or accustic neuroma. Others have reported cases of fatal meningoencephalitis.
The pathogenesis in such cases is probably local oedema due to migration of adult worm wandering into the anterior chamber of the eye ball. It was also seen in pert-orbital tissues, eyelids and into the sub-conjuctival tissues though uncommonly. The other uncommon manifestations included pericardial effusion and acute abdomen.
We are thankful to the Dean, Goa Medical College, Panaji, Goa for his kind permission to publish this paper.