1/23. Lipedematous alopecia: a clinicopathologic, histologic and ultrastructural study.Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick, boggy scalp with varying degrees of hair loss that occurs in adult black females, with no clearly associated medical or physiologic conditions. The fundamental pathologic finding consists of an approximate doubling in scalp thickness resulting from expansion of the subcutaneous fat layer in the absence of adipose tissue hypertrophy or hyperplasia. Observations by light and electron microscopy detailed in this report suggest that this alteration principally manifests by localized edema with disruption and degeneration of adipose tissue. Some diminution in the number of follicles as well as focal bulb atrophy is noted. Aberrant mucin deposition such as that seen in myxedema or other cutaneous mucinoses is not a feature. The histologic findings bear some resemblance to those seen in lipedema of the legs, a relatively common but infrequently diagnosed condition. We present a case of lipedematous alopecia with emphasis on histologic and ultrastructural features. The etiology is unknown.- - - - - - - - - - ranking = 1keywords = adipose, fat (Clic here for more details about this article) |
2/23. Aortocoronary bypass grafting in a child with coronary artery obstruction due to mucocutaneous lymphnode syndrome: report of a case.A four-year-old boy with a myocardial infarct and total occlusion of the right coronary and the left anterior descending coronary arteries due to mucocoutaneous lymphnode syndrome (MCLS). confirmed by selective coronary arteriography, underwent successful double aortocoronary bypass grafting. Patency of the grafts was demonstrated by graft angiography and the improvement of the contractile pattern of the left ventricle was reflected by the increase in ejection fraction from 0.45 to 0.61. This is the first patient successfully treated by aortocoronary bypass grafting for coronary artery obstructive lesions due to MCLS. This experience has demonstrated the feasibility of surgical management in a child with coronary artery obstruction due to MCLS. However, there are unanswered questions regarding the fate of the saphenous vein graft in relation to the growth of a child. Long-term clinical and angiographic follow-ups are mandatory to determine the significance of this mode of surgical treatment for the sequela of MCLS.- - - - - - - - - - ranking = 0.005310569841124keywords = fat (Clic here for more details about this article) |
3/23. yellow nail syndrome in a 10-year-old girl.A 10-year-old girl with yellow dystrophic nails, bronchiectasis, chronic sinusitis and lower-limb lymphedema is presented. The underlying mechanism remains unknown although it has been postulated to be associated with lymphatic abnormalities. To date no causative treatment exists. Our patient was treated with conservative management, including a low-fat diet supplemented with medium-chain triglycerides. Moderate improvement in the lymphedema of the lower extremities was observed. To our knowledge this is the first case of yellow nail syndrome to be treated with diet.- - - - - - - - - - ranking = 0.005310569841124keywords = fat (Clic here for more details about this article) |
4/23. Lipedema complicated by lymphedema of the abdominal wall and lower limbs.We describe a 52 year-old woman in whom lymphedema primarily of the abdominal wall was superimposed on lipedema resulting in an abdomen of enormous dimensions with marked impairment of ambulation. Treatment consisted of preoperative compression of the legs by an external pneumatic device (Lympha-Press) followed by excision of the lymphedematous abdominal fat pad in conjunction with "debulking" of the right leg. The patient illustrates the extremes of lipedema complicated by lymphedema and the technical difficulties associated with its management.- - - - - - - - - - ranking = 0.005310569841124keywords = fat (Clic here for more details about this article) |
5/23. Angiosarcoma associated with chronic lymphedema (Stewart-Treves syndrome) of the leg: MR imaging.Magnetic resonance (MR) imaging findings of two patients with Stewart-Treves syndrome are presented. MR imaging showed edematous changes in the subcutaneous fat and skin masses that proved to be angiosarcomas. MR signal intensity of the tumor was low compared with fat on T1-weighted images and intermediate and heterogeneous on T2-weighted images. In one patient, administration of intravenous Gd-DTPA showed marked enhancement in the early phase, which persisted until the delayed phase. These finding on dynamic MR imaging may reflect the abundant vascular spaces seen in these tumors.- - - - - - - - - - ranking = 0.010621139682248keywords = fat (Clic here for more details about this article) |
6/23. Massive localized lymphedema: additional locations and association with hypothyroidism.We report the second series of a new entity called "massive localized lymphedema in morbidly obese patients" (MLL), recently described in medical literature. Our 6 cases present additional locations as well as an association with hypothyroidism. Huge masses, of longstanding duration ranging from 9 months to 8 years, afflicted the thigh, popliteal fossa, scrotum, suprapubic and inguinal region, and abdomen of morbidly obese adults. Although clinical impressions were generally of a benign process, including lipoma and recurrent cellulitis, the possibility of a malignant neoplasm could not be eliminated. Poorly defined and non-encapsulated, these skin and subcutaneous lesions were most remarkable for their sheer size, measuring 50.6 cm in mean diameter (range, 38-75 cm) and weighing a mean of 6764.5 g (range, 2,060-12,000 g) The overlying skin exhibited the induration and peau d'orange characteristic of chronic lymphedema. Grossly and histologically, a prominent marbled appearance, rendered by fibrous bands intersecting lobules of adipose tissue, simulated sclerosing well differentiated liposarcoma. However, the absence of atypical stromal cells, atypical adipocytes, and lipoblasts precluded the diagnosis of well differentiated liposarcoma. Instead, reactive features, encompassing lymphatic vascular ectasia, mononuclear cell infiltrates, fibrosis, and edema between the collagen fibers, as well as ischemic changes including infarction and fat necrosis, established the diagnosis of MLL. Although the pathogenesis of MLL may be as simple as obstruction of efferent lymphatic flow by a massive abdominal pannus and/or prior surgery, the presence of hypothyroidism in 2 of our patients suggests an alternative pathogenesis. Recognition of this entity by both clinicians and pathologists should avert a misdiagnosis as a low-grade liposarcoma.- - - - - - - - - - ranking = 0.50265528492056keywords = adipose, fat (Clic here for more details about this article) |
7/23. A family with IgA nephropathy and hereditary lymphoedema praecox.immunoglobulin a (IgA) nephropathy is the most common primary glomerulonephritis worldwide. The pathogenesis is still unknown and treatment has not yet been established. Rarely it can be associated with other disorders. Its association with hereditary lymphoedema is not reported before. We report four patients, a 60-year-old father, his two sons and his daughter, with hereditary lymphoedema. The family had nine members and in four of them lymphoedema was evident. The other members had neither lymphoedema nor IgA nephropathy. This is the first report of IgA nephropathy in association with hereditary lymphoedema.- - - - - - - - - - ranking = 0.005310569841124keywords = fat (Clic here for more details about this article) |
8/23. tamoxifen for retroperitoneal fibrosis.retroperitoneal fibrosis is characterised by diffuse inflammatory proliferation of fibroblasts and the deposition of collagen fibrils throughout the retroperitoneum, especially in perivascular sites. This results in blockage of the ureters and other tubular retroperitoneal structures such as the vasculature and lymphatic channels. Left untreated, it typically follows a progressive and fatal course. The condition responds favourably to tamoxifen and several case reports describe an improvement in renal function on this therapy. This case report describes a subjective and objective functional improvement of lymphoedema secondary to retroperitoneal fibrosis with the use of tamoxifen.- - - - - - - - - - ranking = 0.005310569841124keywords = fat (Clic here for more details about this article) |
9/23. Pseudosarcoma: massive localized lymphedema of the morbidly obese.Massive localized lymphedema is a term used to describe a benign overgrowth of lymphoproliferative tissue in morbidly obese patients, which is characterized by fibrotic and edematous fibroadipose tissue. Because of its large size and similar appearance to sarcomas, it has often been termed a pseudosarcoma. patients tend to seek treatment only when the masses reach a sufficient size to alter their activities of daily living or have problems with excoriation or wound breakdown. Resection is indicated in these cases or if there is any question as to underlying malignancy of the lesion. Although recurrence is common, overall prognosis is good, with only anecdotal reports of transformation to angiosarcoma in the literature. We report a patient afflicted with this unique disorder.- - - - - - - - - - ranking = 0.49734471507944keywords = adipose (Clic here for more details about this article) |
10/23. Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia.Pulmonary lymphangiectasia is an uncommon congenital anomaly, and familial occurrence has rarely been reported. We report on two sibs with bilateral pleural effusion/chylothorax and hydrops who died neonatally. One sib required prenatal intrauterine hemithoracic drainage. autopsy confirmed congenital pulmonary lymphangiectasia (CPL) histologically in the first case. Hydrops, characterized as subcutaneous edema and effusions in two or more body cavities, may be due to a variety of factors, but the co-occurrence of CPL in one of these sibs, although rare, supports the notion that chylothorax and hydrops may be caused by structural lesions of lymph channels. Although most cases of CPL are sporadic, the reported sibs support autosomal recessive inheritance, with intrafamilial variability of a lymphatic disorder on a genetic basis. Mutations in vascular endothelial growth factor receptor-3 (VEGFR3) in families with Milroy disease, mutations of FOXC2 in the lymphedema-distichiasis syndrome, and fatal chylothorax in alpha9-deficient mice are potential candidate genes.- - - - - - - - - - ranking = 0.005310569841124keywords = fat (Clic here for more details about this article) |
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