Cases reported "Lipodystrophy"

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1/24. A case of acquired partial lipodystrophy associated with localized scleroderma and undifferentiated connective tissue disease.

    The authors describe a case of a 43-year-old woman affected by acquired partial lipodystrophy associated with localized scleroderma and undifferentiated connective tissue disease. Moreover, they review the literature on pathogenetic mechanisms suspected to be involved in partial lipodystrophy and their association with autoimmune diseases.
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2/24. Two Japanese cases of localized involutional lipoatrophy.

    We present two Japanese cases of involutional lipoatrophy. The first case is that of a 30-year-old woman, who first appeared at our hospital complaining of a localized, well-demarcated depression, approximately 3 x 4 cm in size, normal to slightly erythematous in coloration, on the lateral side of the left upper arm (Fig. 1a). The condition was asymptomatic, and she had noticed this anomaly a month prior to consultation. She received intramuscular injections of corticosteroids of unknown dosage at the affected site for the treatment of allergic rhinitis 4 months prior to her present consultation. The second patient, a 23-year-old woman, appeared at our hospital complaining of a similar macule 4 x 4 cm in size, which she noticed several weeks prior to her most recent consultation. She had no history of injury or injection at the site before the development of the condition (Fig. 1b). She had been under treatment for atopic dermatitis since early childhood and was treated only with topical applications of white petrolatum containing 2% salicylic acid for the past several years. In order to rule out the possibility of acquired partial lipodystrophy associated with localized scleroderma, lupus profundus and the other connective tissue diseases, a histological examination was performed for both patients. Histopathological analysis of the region exhibited a well-defined fat lobule composed of numerous small adipocytes (Fig. 1c) embedded in hyaline connective tissue. edema and dilated capillaries were noticeable in the subcutaneous tissue surrounding the area. Inflammatory cells were not prominent, although mononuclear cells were observed in both patients. No epidermal change was seen in either patient. Direct and indirect immunofluorescence studies revealed no deposits of immunoreactants in the skin of either patient. Immunohistochemical studies with the antibody against macrophage (anti-CD68 antigen; DAKO.) showed that positive cells were scattered around blood vessels and shrunken lipocytes in the subcutaneous tissues (Fig. 1d). Most of these cells in the fat lobules were also positive for mucin stains such as alcian blue. No abnormal findings came to light in the ordinary hematological and blood chemistry examinations of both patients. The autoantibody screening tests using antinuclear, anti-dna, anticentromere, and anti-Scl-70 antibodies were negative in both patients.
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3/24. Soft tissue composition in upper leg lipodystrophy: application of dual energy x-ray absorptiometry.

    Liposuction is a safe procedure for removal of subcutaneous fat in patients with upper leg lipodystrophy. We present a young woman with lipodystrophy of upper leg who underwent ultrasound-assisted three-dimensional suction lipectomy. The aim of the study was to determine the amount of fat to be removed before liposuction and to evaluate soft tissue composition before and after liposuction. At the initial meeting and four months later, body composition parameters were examined by dual X-ray absorptiometry (DXA), which produced a precise assessment of regional fat distribution. The baseline DXA measurements revealed excess of fat tissue for the lateral regions of the right and left legs, and for the area from the waist to hip. After liposuction of established amount of tissue, the same measurements revealed a decrease of fat tissue in all three regions, whereas no significant differences were observed for lean mass. We concluded that the suction lipectomy produced decrease in the fat mass of legs without musculature damage. The DXA technique can be useful in predicting fat removal before suction lipectomy and in estimating changes in soft tissue composition after surgical treatment.
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4/24. Localized involutional lipoatrophy: report of six cases.

    We herein report six cases of localized involutional lipoatrophy, who presented with a depressive plaque on the lateralis of the upper arm. All patients were female, and three had previous injections for allergic rhinitis at the affected sites before the development of lipoatrophy. One patient received intramuscular injection at the affected site when she had a cold. Histological examination showed fat lobules composed of small lipocytes in the hyaline connective tissue. Immunohistochemical examination revealed a number of CD68-positive macrophages in the fat lobules. We consider that localized involutional lipoatrophy is more common than has been reported, and is occasionally induced by local injections.
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5/24. Free gluteal artery perforator flap with a short, small perforator.

    The introduction of supermicrosurgery has led to the development of a new gluteal perforator flap nourished only by a musculocutaneous perforator of the superficial gluteal artery system. This flap has a perforator that is short (3-4 cm in length) and small (less than 1 mm). The successful transference of a free gluteal perforator flap for the coverage of soft-tissue defects in the foot and face in two patients is described in this article. With this flap, deeper and longer dissection for a pedicle vessel is unnecessary, flap elevation time is shorter, thinning of the flap with primary defatting and creation of an adiposal flap with customized thickness for tissue augmentation are possible, the donor site is in a concealed area with minimal donor site morbidity, and application of the flap as a scarred flap for previous operations in the gluteal region is possible. The disadvantages of this flap are necessity of dissection for a smaller perforator and of anastomosis of small-caliber vessels of less than 1.0 mm.
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6/24. Lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.

    An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand dna, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.
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7/24. insulin-induced lipohypertrophy: report of a case with histopathology.

    An 82-year-old woman with type 2 diabetes had been treated with recombinant human insulin for 16 years. She developed large swellings in both sides of her lower abdomen. The masses were soft, painless, and located around her insulin injection sites. Based on the history and clinical features, a diagnosis of insulin-induced lipohypertrophy was made. Total resection revealed that the lesions were composed entirely of fatty tissue. Microscopic examination showed nests of mature adipocytes expanding toward the dermal reticular layer. The hypertrophic adipocytes were twice as large as those from normal subcutaneous areas and contained numerous small lipid droplets. Electron microscopic analysis also revealed a minor population of small adipocytes, suggesting active differentiation or proliferation. Thus, the possible in vivo effects of insulin on adipocytes were clearly observed in this case of insulin-induced lipohypertrophy. To our knowledge, this is the first report of insulin-induced lipohypertrophy with detailed histological examinations.
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8/24. An unusual case with membranous lipodystrophy in a hypertensive patient with transepidermal elimination.

    Membranous lipodystrophy represents a peculiar type of fat necrosis that is present in patients with various types of skin disease. It is characterized by the presence of microcysts and macrocysts and is lined by amorphous eosinophilic material with a crenelated arabesque appearance. These findings have been associated with lupus erythematosus, diabetes mellitus, erythema nodosum, trauma, etc. We report a case of a 43-year-old woman who had a red to purple asymptomatic indurated plaque, approximately seven cm in diameter and on the left arm. She was a chronic hepatitis b antigen carrier and had hypertension for four years. Histopathology of the biopsied lesion showed transepidermal elimination of altered collagen and elastic fibers, as well as membranous lipodystrophy changes. There were hypertensive vascular changes including lymphohistiocytic infiltration around the vascular wall, swelling of endothelial cells, increased thickness of the vascular walls, and narrowing of the lumen. We report a case showing transepidermal elimination with membranous lipodystrophy. We carefully suggest that the secondary phenomenon of transepidermal elimination was associated with membranous lipodystrophy and degenerate connective tissues.
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9/24. Membranous lipodystrophy (Nasu-Hakola disease) with thalamic degeneration: report of an autopsied case.

    An autopsied case of membranous lipodystrophy (Nasu-Hakola disease, NHD) with thalamic degeneration was reported. A 34-year-old Japanese man was diagnosed as having NHD by bone biopsy prior to the onset of clinical symptoms. His maternal grandfather and paternal grandmother are cousins, but this family history is negative for NHD. He developed frontal lobe syndrome at the age of 35 with progressive dementia, and died of acute renal failure at the age of 46. Gross inspection of the brain detected atrophy and softening of the cerebral white matter, predominantly in the frontal lobe. Microscopically, numerous spheroids, predominant fibrillary gliosis with less prominent demyelination "dissociation glio-myelinique" and scanty sudanophilic lipid droplets were observed, indicating the sclerosing type of NHD. An unusual patholgoical finding in this case was selective involvement of the thalamic nuclei with preservation of the other gray matter except for focal cortical necrosis. The topography of the affected thalamic nuclei is similar to that of systemic thalamus degeneration. An association with thalamic degeneration in NHD has not been previously reported. The present case suggests that NHD also affects the thalamus.
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10/24. Localized lipodystrophy with panniculitis: light and electron microscopic studies.

    A 41-year-old woman with localized lipodystrophy histologically showed lymphohistiocytic inflammation within adipose tissue at the periphery and loss of adipose tissue in the center of the lesion. Ultrastructurally, various degrees of lipophagocytosis by macrophages and giant cells were seen, starting with the projection of cytoplasmic processes into the fat droplet and engulfing pieces of the fat droplet which finally were digested. Laboratory investigations revealed a high serum gamma-globulin level and a positive antinuclear antibody, suggestive of a connective tissue disease or autoimmune disease.
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