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Angiosarcoma that develops in areas of chronic lymphedema is also called Stewart-Treves syndrome. It usually appears in areas of lymphedema several years after mastectomy for breast carcinoma. Only 10% of these angiosarcomas occur in areas of chronic lymphedema as a result of another cause. We present a patient with epithelioid angiosarcoma as a rare complication of elephantiasis.
Elephantiasis nostras (EN) is a clinical entity that usually presents as a persistent swelling of the lower extremities. It has been related to recurrent lymphangitis of bacterial origin that causes a fibrosis and thickening of both epidermal and connective tissue. Although very rare, EN has been previously reported in the lips. This is the first case reported in the oral medicine literature that describes EN involving the lips. We describe the clinical features and a differential diagnosis of the lip lesions and a treatment protocol to which this patient has responded. A diagnosis of EN should be entertained in patients with chronically edematous, scaling lip lesions.
A randomized double-blind, placebo-controlled, trial of 5,6 benzo-[alpha]-pyrone (coumarin, '56 BaP') and of diethylcarbamazine (DEC) is being performed on patients with filaritic lymphoedema and elephantiasis over two years, using matched groups. In 169 patients there were significant (1% level) reductions in the amounts of oedema for the patients taking 56 BaP. The excess limb volumes were reduced from 40 to 25% over two years. A similar, but less significant (5% level), improvement was found for the circumference measurements. The rate of reduction was increased when the initial amount of oedema was greater. This therapy, while much slower than many other methods of treatment, does convert a slowly worsening condition into a slowly improving one. There were no significant reductions in the amount of oedema following DEC treatment.
The presence of microparticles of clay is demonstrated in the dermis of the foot in a patient with endemic elephantiasis. The particles are seen to be in the phagosomes of macrophages or in the cytoplasm of other cells. The conducting lymphatic in the subdermal tissue is found to be impermeable to Patent Blue Violet dye and to be fibrosed. The failure to conduct lymph to the node produces a permanent deposit of silica in the dermal tissues; a parallel is drawn with similar deposits in the lung in pneumoconiosis.
Endemic (non-filarial) elephantiasis of the lower legs is a disease of worldwide distribution in which the first signs commonly occur in childhood, at a time when the disease can be arrested and even reversed. Failure to recognize this stage exposes the patient and his family to increasing disability. The distribution, pathology and clinical appearance are summarized with illustrative cases. The management of these cases is outlined. Public health education, especially that of parents, plays a major part in control of the disease.
A case of left facial elephantiasis neurofibromatosa was treated with near-total excision and skin graft. Results were favorable. Repositioning of canthal ligaments, preserving part of relatively normal masseter muscle and primary nasal reconstruction with forehead flap were performed. The skin graft over the myxomatous muscle was successful. Troublesome bleeding was avoided or controlled by incising the normal skin, subperiosteal dissection, hypotensive anesthesia, and chromic catgut suture ligature.
The surgical management of a case of severe scrotal elephantiasis is presented. Excellent functional and esthetic results were achieved by excision of the involved scrotal tissue, and a transverse closure.
Hidradenitis suppurativa is a chronic relapsing infection of the apocrine sweat glands. Its association with penoscrotal lymphedema is not well recognized. A case of massive scrotal elephantiasis associated with chronic hidradenitis of the perineum and scrotum is described. A wide resection of the scrotal mass and perineum was performed, with reconstruction of the perineum and penis carried out using local skin flaps and split-thickness skin grafts. This one-stage treatment yielded an excellent cosmetic and functional outcome.
Nurses in many parts of the United States have limited experience in caring for patients with Kaposi's sarcoma. The severity of the disease can range from a few lesions to extensive cases with fungating, encrusted wounds. This case study describes the wound management of a 43-year-old patient with an advanced case of Kaposi's sarcoma with elephantiasis of the lower extremities. Use of a collaborative care approach resulted in dramatic improvement of this patient's wounds. Although the patient's serum albumin level remained low, no secondary skin breakdown or infection occurred.
Elephantiasis nostras verrucosa is characterized by chronic secondary, non-filarial lymphoedema due to recurrent lymphangitis, dermal fibrosis, and epidermal changes consisting of hyperkeratotic, verrucous and papillomatous lesions. Histologically, there is pseudoepitheliomatous hyperplasia. Therapeutic efforts should aim to reduce lymph stasis, which will also lead to improvement of the cutaneous changes. In this study, rapid disappearance of the hyperkeratotic and verrucous lesions, remarkable flattening of the papillomatous nodules and improvement of lymphoedema occurred in three obese patients treated with etretinate in an initial dose of 0.6-0.75 mg/kg/day for 4-6 weeks. Monitoring of plasma concentrations of etretinate, acitretin and 13-cis-acitretin by HPLC revealed sufficient short-time absorption (4 h) and bioavailability of the drug (30 days; two out of three patients). Long-term maintenance therapy in one patient produced a remarkable improvement in the lymphoedema; another patient relapsed after discontinuation of the etretinate and responded again after this was reintroduced. In the third patient treatment was withdrawn because of an increase in triglycerides, but improvement persisted 6 months later. The clinical side-effects of oral retinoid therapy were moderate and well tolerated.
BACKGROUND: Endemic non-filarial elephantiasis or podoconiosis is a chronic and debilitating geochemical disease occurring in individuals exposed to red clay soil derived from alkalic volcanic rock. It is a major public health problem in countries in tropical Africa, Central America and North India. OBJECTIVE: To estimate the direct and the average productivity cost attributable to podoconiosis, and to compare the average productivity time of podoconiosis patients with non-patients. METHODS: Matched comparative cross sectional survey involving 702 study subjects (patients and non-patients) supplemented by interviews with key informants in Wolaita Zone, southern Ethiopia. RESULTS: Total direct costs of podoconiosis amounted to the equivalent of US$ 143 per patient per year. The total productivity loss for a patient amounted to 45% of the total working days per year, causing a monetary loss equivalent to US$ 63. In Wolaita zone, the overall cost of podoconiosis exceeds US$ 16 million per year. CONCLUSIONS: Podoconiosis has enormous economic impact in affected areas. Simple preventive measures (such as use of robust footwear) must be promoted by health policy makers.
OBJECTIVE: To assess the outcome of the surgical management of severe elephantiasis of male external genitalia. PATIENTS AND METHODS: Twelve patients with gross oedema of their penis and scrotum were managed by excision of the lymphoedematous skin and subcutaneous tissue and a split-skin graft. RESULTS: The cosmetic and functional results were satisfactory in all cases. There was no recurrence and all patients were able to resume sexual activity. CONCLUSION: Surgical excision of affected tissue and split-skin graft is an effective management for severe lymphoedema of the scrotum and penis.
We report 2 unusual gynaecological manifestations of Donovanosis in young tribal aborigines. The first patient presented for medical attention after haemorrhaging from a large pedunculated lesion arising from the left labium minus and majus. This lesion was subsequently confirmed as pseudo-elephantiasis resulting from long-standing Donovanosis of the vulva. The second patient, with initial findings consistent with a diagnosis of advanced cervical malignancy, was referred to the oncology service of a tertiary referral centre. Because of her age and race a differential diagnosis of Donovanosis was considered and subsequently confirmed on special staining (Warthin-Starry) of biopsy specimens. No evidence of malignancy was found in these specimens.
Elephantiasis nostras verrucosa is an unusual and impressive cutaneous hypertrophy that may occur secondary to chronic, severe lymphedema or venous insufficiency, usually of a lower extremity. The condition consists of hyperkeratotic, verrucous, and papillomatous projections. A case is reported in a white woman.
BACKGROUND: Elephantiasis nostras verrucosa represents a rare group of cutaneous changes comprising dermal fibrosis, hyperkeratotic, verrucous, and papillomatous lesions after chronic secondary, nonfilarial lymphedema. There is no standard treatment for this rare cutaneous manifestation. OBJECTIVE: This article describes debridement that is helpful when performed in addition to physiotherapy. METHODS: We present a patient who was treated by shaving the verrucous area with a blade of a freehand knife and by subsequent abrading of the mossy area using a motor-powered grinder. RESULTS: Reepithelization was completed in 2 weeks. A compression stocking was used to treat the lymphedema. Ten months after the operation, we saw no signs of disease recurrence. The result was also aesthetically satisfactory. CONCLUSIONS: Surgery in such case may not always be the best treatment because it does not treat the cause of disease but only treats the cutaneous symptoms. Nevertheless, debridement is a rapid and an aesthetically acceptable form of treatment.
Lymphogranuloma venereum (LGV) is a sexually transmitted disease of the lymph channels caused by Chlamydia trachomatis. Esthiomene, a rare late manifestation of LGV, is a primary infection affecting the lymphatics of scrotum, penis, or vulva. The male genitalia are affected less commonly by esthiomene, but we report a male patient who presented with inguinal syndrome, penoscrotal elephantiasis, along with enlargement of femoral lymph nodes.
Elephantiasis of the scrotum is the terminal stage of persistent refractory lymphedema. Its debilitating functional and cosmetic effects have significant psychological, emotional and social consequences for the affected patient. The causal relationship between etiology and recurrent disease is illustrated here by 2 cases. Therapy options are also discussed. Primary therapeutic success is determined by radical surgery, since chronic inflammation and chronic oedema mutually foster one another. Metabolic stability, sanitization of infected cutaneous areas and the prophylactic administration of antibiotics are essential to ensure that the treatment of recurrent erysipelas is successful in the long term.
Elephantiasis nostras, the result of chronic lymphedema, is characterized by marked edema of the affected extremity with a thickened, verrucous, pebbly appearance of the skin. The pathogenesis is thought to be related to fibroblast proliferation following impaired lymphatic drainage, leading to fibrosis and further restriction of lymph drainage with progressive edema. A case report of a patient with massive chronic lymphedema of her feet is presented.