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Elephantiasis nostras: an eight-year observation of progressive nonfilarial elephantiasis of the lower extremity.

An eight-year follow-up of a progressive case of nonfilarial elephantiasis affecting the lower extremity and a review of the literature is presented. The natural history and management of this disorder are discussed, together with recommendations to assist the clinician in early identification an treatment. Elephantiasis nostras is characterized by chronic enlargement of a limb, resulting from lymphatic blockage secondary to recurrent attacks of streptococcal lymphangitis. Each subsequent attack results in greater edema of the limb. Without appropriate intervention the cycle continues until the deformity is greatly exaggerated. Control of edema an infection is important in preventing the recurrent lymphangitis that will eventually result in grotesque enlargement of the limb. Photographic documentation and serial circumferential limb measurements are recommended to document progression of the disease and effectiveness of treatment.

Elephantiasis

Treatment of elephantiasis in a community with timorian filariasis.

To elucidate the issue of lymphoedema and elephantiasis in our previous reports, all data on the two clinical conditions have been reviewed and analysed. The various characteristics of the swelling are described and factors that may influence the outcome of treatment with diethylcarbamazine (DEC) analysed. The results showed that all cases with lymphoedema could be treated with DEC, irrespective of the size of the swelling, and that in most cases the swelling disappeared within one year. On the other hand, it required at least two to four years for most swelling to disappear in people with elephantiasis. Elephantiasis of the arms was easier to treat than of the legs. Bilateral elephantiasis of the legs were more difficult to treat than unilateral elephantiasis. Elephantiasis of less than three to five years' duration were easier to treat than that of longer duration. Individuals with a higher grade of elephantiasis were more difficult to treat than those with a lower grade of elephantiasis. The age and sex of patients did not influence the outcome of treatment.

Adolescent

Endemic non-filarial elephantiasis in Iringa District, Tanzania: a study of 30 patients.

Endemic non-filarial elephantiasis has not yet been described in the central tableland of Tanzania. We report the results of a clinical study in Tosamaganga Hospital, located in the middle of Tanzania (Iringa District). 30 patients with elephantiasis of the lower limbs were studied parasitologically (by blood smears) and clinically. Inguinal lymph nodes were removed from 10 patients and histologically examined. The mineral content of soil samples collected from 4 different areas of the region was assayed by X-ray fluorescence. The clinical, histological, parasitological and epidemiological data prompted us to conclude that in these patients elephantiasis was not of the filarial type, and that endemic non-filarial elephantiasis is present in the Central District of Tanzania.

Adolescent

[Surgical treatment of elephantiasis of the upper extremities].

According to the author' data elephantiasis of the upper extremities was observed in 13 (6.2%) among 215 patients, operated upon for elephantiasis of the extremities. There were 3 males and 10 females. The patients' age ranged from 3 to 56 years. Five patients suffered elephantiasis of the upper extremity since their birth, 8 patients showed acquired elephantiasis, in particular after mastectomy and operative procedures in the axillary region. Late results of surgical treatment proved to be quite satisfactory.

Adolescent

The association of endemic elephantiasis of the lower legs in East Africa with soil derived from volcanic rocks.

Endemic elephantiasis of the lower legs in Ethiopia, which reaches a maximum of 86-7 per 1,000 adults in affected areas, is related to the distribution of red clay soil derived from volcanic rocks, particularly basalt. Prevalence falls rapidly on leaving these areas. This observation has been tested in regions of non-filarial elephantiasis reported in Kanya and north-western Tanzania and further investigated in volcanic areas of Rwanda where the disease had not previously been reported. The same relationship is found to occur in these areas. The limitation to the lower legs of the barefooted section of the farming community suggests that the aetiological factor or factors enter by the feet. The occurrence at high altitude (over 1,200 metres) is noted and the predominance of basalt or basalt-like lava in each case is considered significant. The altitude governs rainfall and temperature and thus governs the type of soil produced. The soil produced from these rocks is rich in colloidal iron oxide, alumina and silica, to which a number of metallic ions are adsorbed. This soil is a reddish-brown clay which, when wet, is strongly adherent to the skin. The derived ions are known to be toxic to human tissue and absorption through intact human skin has been shown to occur experimentally. It is suggested that absorption of these irritants through the bare feet is responsible for the irreversible damage to the lymphatic channels. The present studies support the hypothesis that "high-altitude" elephantiasis of the lower legs in East Africa is a geochemical disease.

Elephantiasis

[Elephantiasis of the external genitalia in children].

This study presents an experience with diagnosis and management of external genital elephantiasis in 18 children in the age range of 3 to 15 years. All patients underwent surgical treatment. The type and scope of surgery depended on the extent and severity of elephantiasis. A total of 21 operations were done. Long-term results were evaluated in these 13 patients at 1 to 22 years. The evaluation included physical appearance of the genitalia, scars, secondary deformities, penile and testicular size, the presence of erections and menstruation. Adults of mature ages were evaluated for fertility and reproductive status. Long-term results were good in all 13 patients. This analysis suggests that early radical surgical treatment of children with congenital elephantiasis of the external genitalia enables a normal anatomic and functional genital development and averts severe local and systemic disorders.

Adolescent

[Filarian elephantiasis in French Polynesia (Wuchereria bancrofti var. pacifica). II. Biological aspects].

Realized in French Polynesia among 274 patients with elephantiasis, this survey studied the microfilaremia, the eosinophily, the immunoglobulin titers and the antifilarian antibodies (done by passive hemagglutination) for Wuchereria bancrofti var. pacifica. Patients with elephantiasis seldom have circulating microfilariae in their blood. Hypereosinophily is frequent but rarely high. It is similar to the one patients with microfilaremia. There is a trend towards neutropenia during lymphangitic crisis that occur on an elephantiasis limb. The IgE titer is clearly increased, the IgG one is lesser elevated. The mean values are identical to those encoutered among microfilariae asymptomatic cariers. On the other hand the serodiagnosis is more frequently positive among elephantiasic patients.

Adult

The site of lymphatic blockade in endemic (non-filarial) elephantiasis of the lower legs.

1. The femoral lymph nodes of 13 patients with endemic (non-filarial) elephantiasis of the lower legs are compared with those of 13 non-elephantiasic adults from the same area. 2. Features characteristic of the disease include reflux of darkly-staining lymphocytes into the afferent lymphatics, crowding of the small lymphocytes into the sinuses with sheets of similar cells in the periphery of the node. The phenomena occur in relation with endolymphangitis of afferent lymphatics in some cases. 3. The picture closely resembles that produced experimentally in animals by occlusion of the incoming lymphatics and is interpreted as indicating delay in, or absence of, transport of the lymphocytes through the node into the central circulation (Gowans cycle). In elephantiasis, the loss of lymph-flow results from endolymphangitis of the afferent lymphatics. 4. The presence of microgranulomata and of birefringent particles was also noted but is not discussed here. 5. The present study indicates that the lesion responsible for the irreversible elephantiasis of the lower legs is in the distal lymphatics.

Adult

Filarial lymphoedema and elephantiasis of lower limb: a review of 44 cases.

Twenty patients with early lymphoedema due to filariasis and twenty-four patients with elephantiasis of the lower limb were subjected to lymph nodovenous shunt (LNVS) and Charles' operation respectively. In 62 per cent of cases subjected to LNVS operation, there was a rapid relief of lymphoedema followed by slow reduction, 24 per cent had slow and gradual reduction and 14 per cent had very slight reduction in volume and circumference. Patients subjected to Charles' operation had immediate volume and circumference reduction and skin graft 'take' was 88 per cent. There were no operative and a few minor postoperative complications in both the procedures, infection being most notable in those who had undergone Charles' operation. The hospital stay was 7-10 days in LNVS and 21-36 days in Charles' operation. There was no mortality. The long-term results of the present study in terms of volume and circumference reduction, skin condition and freeness of joint movement were encouraging. It is concluded that, while excisional surgery such as Charles' operation becomes necessary for late states of lymphoedema which have progressed to elephantiasis, nodovenous shunt alone is sufficient to relieve early stages of lymphoedema due to filariasis.

Adolescent

Microlymphaticovenous anastomosis for treating scrotal elephantiasis.

Scrotal elephantiasis can be physically disabling and psychologically distressing to the victim. Ablative procedure has been used in its treatment and has achieved limited success. The authors developed a microlymphaticovenous procedure to treat elephantiasis of the scrotum and applied it clinically in three patients. The immediate and long-term (13-24 months) results have been very satisfactory. The scrotum size was dramatically reduced to a nearly normal level, and subjective symptoms and objective signs were improved. The operative techniques are described, the three case histories are illustrated, and the advantages of microlymphaticovenous anastomosis, the selection of patients, and the factors required for success of the surgery are discussed.

Adult

The possible role of soil particles in the aetiology of non-filarial (endemic) elephantiasis: a macrophage cytotoxicity assay.

A link between the incidence of non-filarial elephantiasis and tropical red clay soil has been suggested after epidemiological surveys in Ethiopia and other African countries, although the mode of action of these soils in disease induction is unknown. We have thus investigated the physical composition of soils from both endemic and non-endemic areas of Ethiopia and their effect on the viability of macrophages using in vitro systems. Endemic and non-endemic soils were avidly phagocytosed by the macrophages. Cell shape was seen to change over a 144-hour period, the cells changing from approximately circular to spindle or stellate shape. Both groups of soils were cytotoxic towards the macrophages. Greatest cell death occurred during the first 48-hour period although some cells were viable after 96 hours. The soil groups could be divided by assessing their contents of silicon and aluminium, as well as observing the number of sub-2 micron particles they contained. The present study gives support to the epidemiological studies implicating soils as the cause of non-filarial elephantiasis.

Aluminum

Elephantiasis nostras 1984.

Elephantiasis nostras is a rare condition that usually presents as a persistent swelling of the lower extremity secondary to recurrent lymphangitis. We present a patient, originally referred for "angioneurotic edema of the lip," who presented with a history of several months of persistent swelling of the upper lip. There was probable evidence for recent infection of the lip that is consistent with the diagnosis of elephantiasis nostras. This disease should be included in the differential diagnosis of chronic persistent angioedema of the lip.

Adult

Angioedema, elephantiasis nostras, and cheilitis granulomatosa.

Angioedema of the face is a common entity seen by the allergist, but other less common conditions should also be considered in the differential diagnosis of facial swelling. Elephantiasis nostras is a rare condition that can result in persistent swelling of the lips secondary to recurrent attacks of lymphangitis caused by bacterial infection. Cheilitis granulomatosa is a disorder that may also present with lip swelling. We present a patient with persistent swelling of his lips who was referred to our service to consider an allergic cause. The clinical findings of persistence of the swelling without improvement after therapy with corticosteroids and antihistamines are considered consistent with elephantiasis nostras or cheilitis granulomatosa. The histopathologic findings in this case revealed epithelioid cell granulomas, which are consistent with cheilitis granulomatosa. These two disorders should be included in the differential diagnosis of localized swelling of the face.

Adult

Buphthalmos and progressive elephantiasis in neurofibromatosis. A report of three cases.

Two cases of buphthalmos and regional gigantism in peripheral neurofibromatosis (Von Recklinghausen disease) are described. The ocular hypertension was detected at birth and thereafter the facial hemihypertrophy, café-au-lait spots and neurofibromas. One case presented a progressive facial elephantiasis without buphthalmos. The evolution in neurofibromatous elephantiasis of these three cases is discussed.

Child

Endemic elephantiasis of the lower legs in Rwanda and Burundi.

The distribution of non-filarial elephantiasis of the lower legs in Rwanda and Burundi in Central Africa has been studied in order to test the previous observation of an association between the disease and the volcanic rocks and soils on which the people live. Using the method of market counts, a total of 26,602 adults were observed in 23 markets; 189 had elephantiasis. In addition, 264 cases were observed in specially arranged clinics and a further 77 while travelling. Prevalence per thousand adults varied from 0.0 to 20.7. It was noted that the areas of high prevalence corresponded with the areas of volcanic lava. A differential market count at the edge of the red soils in two of these areas showed a raised prevalence among people living on these soils, similar to that observed in Ethiopia. The observations confirm that the disease occurs in barefooted people who dig in soil developed from volcanic rocks, under tropical conditions, at altitudes around 1500 m. It is suggested that the high proportion of iron and other transitional metals may be important as irritant or toxic to the lymphatic vessels of the legs after absorption.

Adult

Elephantiasis nostras.

Elephantiasis nostras is characterized by edema, skin fibrosis, and massive enlargement of a body part. Lymphatic obstruction, most commonly due to surgery, radiation, infection, or neoplasms, is important in its pathogenesis. The diagnosis of elephantiasis nostras can often be made based on the clinical findings, but examination of tissue may be helpful to rule out associated conditions, especially malignancies. Mainstays of therapy are elevation, use of pressure devices, and administration of antibiotics. Although medical and surgical treatments are limited in their value, pneumatic pumps are effective in refractory cases.

Adult

[Microsurgical therapy of scrotum elephantiasis].

Scrotoplasty has been used in the treatment of scrotal elephantiasis but it is most traumatic and achieved unsatisfactory results. The author developed a microlymphatico-venous procedure to treat elephantiasis of the scrotum and applied it clinically with good results. The scrotal size was immediately reduced to a nearly normal value. The indication, types of procedures and advantages of the operation are presented.

Adult

[Filarian elephantiasis in French Polynesia (Wuchereria bancrofti var. pacifica). Health study on 274 subjects. I Epidemiological and Clinical aspects].

Twenty-five years after the fight started against Wuchereria bancrofti var. pacifica in French Polynesia and which was based upon mass chimiotherapy by diethylcarbamazine (Banocide), a survey realized in 1975 and 1976 among 274 patients with elephantiasis allows us to give precise details of the local clinical and epidemiological aspects. Since 1950 the number of clinical cases varies along with the parasitological incidence, but with a certain delay which is explained by the late and irreversible elephantiasis characteristics. The overall prevalence is actually low. However the occurrence of new cases, although very rare, is still possible. Among sick patients the lymphangitic crisis frequency whose aspect lead to evoke a bacterial participation, is low. Women are less frequently and less severely involved than men. Localisations are essentially restricted to the lower limbs. Several limbs are involved in approximately 50 0/0 of the cases. The scrotum involvement is rare, the one of the breast is found only once. Among men we can notice a 33 0/0 incidence of associated hydroceles. A past history of chyluria is not rare.

Adult