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Elephantiasis nostras.

Elephantiasis nostras is one of the most striking yet, at the same time, one of the most poorly understood dermatologic entities. We present a report on two patients with this disorder, each of whom had a marked enlargement of a lower extremity following recurrent attacks of infection and/or ulceration. In one case obesity and chronic cardiac disease may have been aggravating factors. In the second, the enlargement and deformity was severe enough to require amputation of the foot and lower leg. Study of these and other cases may lead to further insight into the pathogenesis of elephantiasis nostras.

Amputation, Surgical↗

Cutaneous metastatic carcinoma and elephantiasis symptomatica.

An elderly man had elephantiasis of a lower extremity that was partially covered with verrucose papules, but also had sharply delineated islands of normal-appearing skin. Biopsy showed cutaneous metastases to be responsible, at least in part, for the clinical appearance. Patients with elephantiasis with "skipped areas" should be suspected of having cutaneous metastatic malignant neoplasms.

Adenocarcinoma↗

Elephantiasis nostras verrucosa.

Elephantiasis is an uncommon disease in the United States. We present a case of nonfilarial elephantiasis nostras verrucosa in a 14-year-old girl. Most commonly, the upper and lower extremities are affected; the disease, in this patient, was confined to the buttock. The patient has done well after excision and primary closure. The patient is currently undergoing pressure therapy 1 year after surgery. She has no evidence of recurrence. We present this case and review the literature.

Adolescent↗

[Operative therapy of elephantiasis penis by skin transplantation (author's transl)].

From 1960 to date, there have been four cases of elephantiasis penis in the urologic clinic of the Katharinenhospital in Stuttgart, which were operated on by skin transplantation. The causes of elephantiasis penis are discussed, and the surgical procedure is presented in detail. Finally, the outcome as far as appearance and function are concerned, is discussed.

Adult↗

[Elephantiasis of the vulva of an unclear etiology: case report].

A patient with vulvar elephantiasis, which had been developing since 1985 when biopsy was first performed showing only signs of chronic inflammation, is described. From 1985-1987 (when she was first admitted to the hospital) microbiological analyses were negative and antibiotic therapy was administered without success. At the first admittance abscess of the left Bartholin's gland and furunculosis of vulva were diagnosed. Serological tests to agents that usually cause vulval infections with elephantiasis were negative, and microbiological analyses revealed mixed bacterial flora. Biopsy showed again only nonspecific chronic inflammation. The patient received ampicyllin, oxytetracyclin and doxycillin. She did not return for the control until 1993. At that time the vulvar mass reached 16:13:10 cm and was surgically removed. The histological picture showed chronic granulomatous inflammation. The results of microbiological and serological tests were again the same as before.

Elephantiasis↗

Elephantiasis nostras verrucosa.

Elephantiasis nostras verrucosa is a rare, chronic, deforming disorder characterized by hyperkeratosis and papillomatosis of the epidermis with underlying woody fibrosis of the dermis and subcutaneous tissue. Chronic lymphedema, either congenital or secondary to infection, surgery, radiation, neoplastic obstruction, obesity, portal hypertension, or chronic congestive heart failure, plays a pivotal role in the pathogenesis. Without appropriate intervention, the slowly progressive cutaneous changes will culminate in massive and grotesque enlargement of the affected body region. In the following case report, the natural history and the management of elephantiasis nostras are discussed.

Diagnosis, Differential↗

Filarial elephantiasis among Haitian women: social context and behavioural factors in treatment.

Few studies have addressed the social and behavioural aspects of lymphatic filariasis. The research reported here investigated the ethnographic context of filarial elephantiasis among women in Léogane, Haiti, and focused on explanatory models of the illness, the impact of the disease on women's lives, and the difficulties patients experienced in following a therapeutic regimen provided at a local hospital. Qualitative data were collected through focus group and individual interviews and direct observation of patients enrolled in the treatment programme. Results indicate that traditional understanding and treatment for the disease are prevalent in the community, although biomedical explanations are gaining credence as a consequence of long-term filariasis control activities in this area. Women's lives are substantially burdened both socially and economically by the physical impairment of elephantiasis, most notably in the loss of income due to restrictions on mobility. The degree of social discrimination encountered varies by the timing of onset of symptoms in the life course. Difficulties encountered with the physical therapy regimen included maintenance of the compressive bandage and availability of suitable foot wear. Similarities between these findings and those reported for other parts of the world are noted. Recommendations from the study cite the need for community education and peer support activities to provide a knowledge base and support structure for current and future intervention programmes.

Female↗

Donovanosis (granuloma inguinale) with vulval pseudo-elephantiasis.

A 24 year old Melanesian female from the Eastern Highlands of Papua New Guinea presented with chronic painless ulcers of the external genitalia, anus and thighs of many years' duration and marked left labial hypertrophy for one year. Scrapings from the ulcers contained large foamy mononuclear cells in which Donovan bodies were demonstrated. The ulcers responded to tetracycline therapy however she absconded after three weeks before surgical correction of the labial pseudo-elephantiasis could be carried out. Vulval pseudo elephantiasis of the magnitude present in this case is rarely seen in Papua New Guinea.

Adult↗

[Surgical therapy of scrotal edema in elephantiasis congenita hereditaria (Meige type)].

The underlying cause of primary lymphedema is a malformation of the lymph vessel system. Secondary lymphedemas can be due to infections, recurrent inflammation, hypoproteinemia, tumors, operations, or irradiation. As a reaction to persistent edema and interstitial macromolecules, fibrosis occurs. Recurrent inflammations of the indurated and edematous tissue are clinically impressive. The massive form of the scrotal lymphedema leads to painful tautness and sexual dysfunction. A concurrent penile edema can cause dysuria. The deformity of the affected extremities and organs not only leads to restriction of mobility but also to psychological stress due to the disfigurement, even as far as to social deprivation. We report on a surgical technique for treating pronounced scrotal edema by resection and neoscrotal reconstruction using ventral pedunculated scrotal skin flaps in cases of congenital hereditary elephantiasis of the Meige type.

Elephantiasis↗

Magnetic resonance imaging of elephantiasis neuromatosa.

We present the case of a 43-year-old man with neurofibromatosis type 1 who developed elephantiasis neuromatosa of his left leg. The gross limb enlargement was extremely disfiguring, and resulted in such severe disability that he was only able to walk a very short distance using crutches. Previous debulking procedures had resulted in massive blood loss, and prior to attempting further surgical intervention MRI studies were requested. Taking advantage of the excellent tissue characterisation and multiplanar imaging capabilities of MRI, we were able to assess the extent of soft tissue and osseous involvement. The use of recently developed MR angiographic sequences enabled us to non-invasively provide detailed images to assess the relationship of the lesions to the major vessels, as well as the vascular supply and angiographic features of the lesions themselves. This article describes our MRI-based findings, which precluded debulking surgery in this unusual manifestation of neurofibromatosis.

Adult↗

Silica and silicates in femoral lymph nodes of barefooted people in Ethiopia with special reference to elephantiasis of the lower legs.

Electron microscopy of femoral lymph nodes of barefooted Ethiopians show the presence of numerous particles of colloid-size and electron-density in the lysosomes of the macrophages in the gland. On diffraction analysis, the particles are found to be amorphous. Elemental microanalysis of the X-ray spectrum indicate the predominance of Si, Al and Fe. In elephantiasics some particles contain silicon alone, presumably silica. The distribution of the Al/Si ratios of the particles in subjects with elephantiasis of the lower legs show a difference from that of non-elephantiasics which is statistically "highly significant" at p less than 0.001. The importance of this as a possible aetiological factor in the disease is discussed. The hypothesis that the disease is discussed. The hypothesis that the disease is a silicosis of the peripheral lymphatics of the lower limbs is supported by the present study.

Aluminum↗

Filarial elephantiasis in French Polynesia: a study concerning the beliefs of 127 patients about the origin of their disease.

127 patients from Tahiti who were suffering from elephantiasis were interviewed about their opinion of the origin of their disease. Ancestral beliefs are still widely held even after 25 years of antifilarial campaigns which have resulted in a drastic decrease in endemicity with almost no clinical incidence. It is disappointing that the responsibility of mosquitoes is denied by a majority of patients. The explanations are to be found in the unusual evolution of this disease and in the small importance attached to sanitary education.

Adult↗

The effects of silica on lymph nodes and vessels--a possible mechanism in the pathogenesis of non-filarial endemic elephantiasis.

Non-filarial tropical elephantiasis, which occurs in certain volcanic areas of the world, has been postulated to be an obstructive lymphopathy due to the fibrogenic effects of silica absorbed through the plantar skin of bare-footed people. Animal experiments involving the direct intralymphatic injection of fine silica particles have been carried out in order to assess the extent to which this substance can engender lymphatic obstruction and to determine its main site of action. Intralymphatic silica provoked an immediate and intense macrophage reaction with later fibrosis both within lymph vessels and to a lesser extent within lymph nodes. Lymphography indicated that the consequent obstruction resulted more from the effects of silica on vessels than on nodes.

Animals↗

Reconstruction of male external genitalia with elephantiasis.

The surgical rehabilitation of a patient with severely deformed and functionally disabled genitalia due to long-standing lymphedema and infection is described and illustrated. The term "elephantiasis" has been applied to such gross lesions. Appropriate surgery to the penis, scrotum, and perineum has yielded a gratifying result. The principles and techniques of the surgical approach in such an undertaking are presented and discussed.

Axilla↗

Congenital lymphangiectatic elephantiasis.

A case of a child with a rare giant congenital cavernous lymphangioma giving rise to elephantiasis of the left lower extremity is presented. Plain radiographs revealed underlying bone lesions and magnetic resonance (MR) imaging accurately defined extension of the process into the pelvis and retroperitoneum.

Child↗

Idiopathic scrotal elephantiasis.

Scrotal lymphedema (scrotal elephantiasis) is a condition that has historically been described in areas endemic to filariasis. We present a unique case of a 22-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and scrotal reconstruction.

Adult↗

Elephantiasis of the penis and scrotum. A review of 350 cases.

Three hundred fifty patients with elephantiasis of the penis and scrotum were surgically treated by excision of all elephantoid skin and subcutaneous tissues. Before attempting to excise the extensive elephantoid tissues, the spermatic cord and testes with their coverings were taken out by two small lateral incisions, which made the operation easier and less time-consuming and minimized the complications. The skin of the scrotal neck was found to be the best for reconstruction of the scrotum to accommodate the two testes. The fascial penis is an ideal bed for intermediate split-thickness skin grafts. There was mechanical improvement in the physical disability, restoration of potency and sexual habits, and reduction of mental anguish.

Adult↗

Reduction perineoplasty for scrotal elephantiasis: a method of surgical reconstruction of the penis and scrotum.

Scrotal elephantiasis can be insidious in onset, idiopathic as to cause and debilitating in progress. The massive scrotal enlargement results in a sac-like structure that first envelopes and then completely covers the penis, testes and the regional spermatic cord structures, causing loss of topographic identification. The urethra leads into a stratified, squamous epithelial-lined canal, which traverses the edematous subcutaneous tissue to open irregularly at the inferior aspect of the scrotal bag. The difficulty of acquiring sustained periods of cleanliness, the incontinence of urine despite an intact urethral sphincter, the unesthetic gross appearance and the loss of libido and mechanical copulation function are the significant symptoms and signs that indicate the extent of the debility. A form of treatment that would alleviate this distressful state and would be feasibly applicable should be worthy of critical consideration. Therefore, a case of a reduction scrotoplasty (reduction perineoplasty) for idiopathic lymphedema of the penis and scrotum is presented.

Drainage↗