| The most characteristic lesions of neurofibromatosis are the extremely large plexiform neurofibromas involving an entire extremity, which give rise to the condition known as elephantiasis neuromatosa. In this article, we present a patient who was diagnosed as elephantiasis neuromatosa with Becker's melanosis clinically and review the literature briefly. ( info) |
| 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. ( info) |
3/64. Elephantiasic pretibial myxedema: insight into and a hypothesis regarding the pathogenesis of the extrathyroidal manifestations of Graves' disease. The basis for the extrathyroidal manifestations of Graves' ophthalmopathy (GO) and dermopathy are not well understood. We describe immunohistochemical studies on the skin of a patient with an extreme, elephantiasic form of Graves' dermopathy that developed after periods of prolonged standing with dependent edema. Excision of part of the lesion with subsequent skin grafting from a normal donor site resulted in recurrence of the disease at the original site as well as in development of disease at the donor site. A murine monoclonal antibody reacted with the thyrotropin receptor (TSHR) or a cross-reacting protein in fibroblast-like cells in the patient's upper dermis and, surprisingly, with dermal cells from unaffected individuals. The patient's dermis containing lymphoid follicles comprising B cells and CD3 , CD4 T cells, with few CD8 T cells. CD21 cells (most likely follicular dendritic cells) were also present in the dermis. Based on past and present observations, we raise an unifying hypothesis to explain the diverse extrathyroidal manifestations of Graves' disease and their apparent lack of association with TSHR autoantibodies. As opposed to the present concept that these phenomena relate to site-specific properties on preadipocytes or fibroblasts, we suggest that clinically evidence GO and dermopathy are primarily caused by local factors (particularly in the orbit) superimposed on a systemic, low-grade connective tissue inflammation. ( info) |
4/64. Recurrent massive subperiosteal hematoma in a patient with neurofibromatosis. The authors report the case of a 13-year-old neurofibromatosis (NF-I) patient who suffered a blunt trauma in 1993. The diagnosis of subperiosteal hematoma was made. The pathogenesis of subperiosteal hematoma is discussed. ( info) |
| The authors report the case of a 13-year-old patient with neurofibromatosis (NF-I), who suffered blunt trauma to the left tibia in 1993. The diagnosis of subperiosteal hematoma was made. Treatment consisted of temporary rest. There was a recurrence in 1996, and the subperiosteal hematoma was drained. In 1997, a shortening osteotomy of the left tibia was performed. However, massive gigantism with elephantiasis of the left leg remained, causing a serious functional and cosmetic problem. In 1999, the leg was amputated above the knee. The literature is reviewed and 7 case reports are compared. The pathogenesis of subperiosteal hematoma is discussed. ( info) |
| Options in Practice presents different management approaches to the same clinical situation. You are invited to submit a brief case description, including the specialty nursing care provided, and several glossy color photographs of the clinical situation. The case material will then be sent to another wound, ostomy, or continence care nurse, who will also address management concerns. Alternative solutions to difficult wound, ostomy, or incontinence clinical situations will be published. ( info) |
7/64. Elephantiasic pretibial myxedema: a novel treatment for an uncommon disorder. Pretibial myxedema is a known manifestation of Graves' disease. Much less common is the elephantiasis nostras variant, which is often refractory to treatment. We therefore elected to try a new therapy, often used for the management of chronic lymphedema, called complete decongestive physiotherapy. After 6 weeks of intensive treatment, our patient lost 37 pounds and had reduced her edema volume by 47%. Her skin softened with decreased lymph seepage and she became mobile for the first time in years. At a 2-year follow-up visit, she exhibited sustained improvement. This case demonstrates that complete decongestive physiotherapy can provide effective, long-term control of this disease process. We suggest that complete decongestive physiotherapy be considered in patients with severe forms of pretibial myxedema, as well as those with refractory lymphedema. ( info) |
| Genital elephantiasis (esthiomene), which is the dramatic end-result of lymphatic obstruction, is rather rare. Although mainly associated with filariasis and sexually transmitted diseases, such as lymphogranuloma venereum and donovanosis, it could also be an uncommon complication of tubercular lymphadenitis, a common infection in tropical countries. We report a rare case of a 32-year-old Indian female in whom genital elephantiasis occurred as a complication of tubercular lymphadenitis. ( info) |
9/64. Congenital elephantiasis-like lymphangiomatosis of a lower limb. The case of a newborn girl with a rare, giant, congenital, tissue lymphangioma giving rise to elephantiasis of the right lower limb is presented. The different imaging methods, especially magnetic resonance imaging, showed no extension of the lesions into the deep structures. At the age of 2 years, the child underwent a roentgenographic skeletal survey, which revealed osteolytic lesions in the femurs and the right tibia. There was no clinical evidence of systemic involvement. The place of this affection among the different lymphatic malformations was discussed and the diagnosis of elephantiasis-like lymphangiomatosis of the limb, an extremely rare disorder, has been retained. Early surgical reduction was performed, followed by application of a pressure dressing. Five years later the result remains satisfactory, but the excision of a persistent fluid-filled pouch around the knee will probably be necessary in the future. ( info) |
10/64. Symptomatic skeletal muscle metastasis and elephantiastic lymphedema in a patient with recurrent ovarian carcinoma. BACKGROUND: Distant tumor metastases in ovarian cancer patients in striated muscles are extremely rare. So far, only one case of metastatic but asymptomatic spread to striated muscle has been described. CASE: A patient with recurrent ovarian carcinoma presented with a gluteal mass 1 week following optimal debulking surgery. CT and MRI initially suggested muscle necrosis or intramuscular bleeding. Increasing pain and massive lymphedema led to immobilization and clinical deterioration. Repeated ultrasound and CT-guided biopsy confirmed the diagnosis of skeletal muscle metastases. CONCLUSION: This is the first case description of distant skeletal muscle metastasis as the leading clinical symptom of late stage ovarian carcinoma. It reveals possible limitations of CT and MRI as diagnostic tools for early identification of striated muscle metastases. ( info) |