Cases reported "Necrobiosis Lipoidica"

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1/9. necrobiotic xanthogranuloma without periorbital involvement: an ultrastructural investigation.

    A case of necrobiotic xanthogranuloma without typical periorbital involvement is described at the ultrastructural level. The patient, a 58-year-old Italian man, presented in 1995 with a brief history of nodulo-papular lesions commencing on the lower limbs, and mild paraproteinemia. During 6 years of follow-up, anemia, neutropenia with marked lymphopenia, and increased ESR were found, while serum cholesterol and triglyceride levels decreased from hyper to hypo values. Systemic diseases, such as diabetes, malignancy, or extracutaneous lesions, often associated with NXG, have not developed. Conventional histology was distinctive for NXG, and immunohistochemistry confirmed that dermal histiocytes were not of Langerhans cell lineage. At ultrastructure, regeneration and degeneration ("regen-degen") features were observed in some individual deep dermal histiocytes, which have not been previously documented in the literature. Identification of giant histiocytes showing 'regen-degen'' aspects might prove to be a useful ultrastructural diagnostic marker for NXG.
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2/9. Annular elastolytic giant cell granuloma causes an irreversible disappearance of the elastic fibres.

    A 67-year-old man presented with grouped red papules with a smooth surface coalescing to relatively well-demarcated plaques on his left thigh, in the axillae and on the lateral parts of the trunk. The plaques were growing slowly, and the older ones had a frilled surface. A skin biopsy showed a zone of disappearance of the elastic fibres with a rim of giant multinuclear cells with fragments of the elastic fibres in their cytoplasm. This finding is typical of annular elastolytic giant cell granuloma (AEGCG). After corticosteroid therapy, the inflammation resolved, causing the frilled surface of the lesions due to the disappearance of the elastic fibres. Remission of the skin lesions lasted for 1.5 years. A second skin biopsy taken from the site of the previous lesion showed the absence of the elastic fibres, thus their phagocytosis was irreversible.
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3/9. necrobiosis lipoidica diabeticorum with cholesterol clefts in the differential diagnosis of necrobiotic xanthogranuloma.

    The histopathologic findings in 331 cases of necrobiosis lipoidica diabeticorum seen during a 50-year period were reviewed. Three cases showing cholesterol cleft formation were found. All 3 cases were associated with severe diabetes mellitus. The differential diagnosis of importance is necrobiotic xanthogranuloma. Common features included extensive hyaline necrobiosis and foreign-body giant cells. Atypical and Touton-type giant cells are more common in necrobiotic xanthogranuloma. Vascular changes in necrobiotic xanthogranuloma may include granulomatous involvement of muscular walls with thrombosis. Explanations for cholesterol cleft formation are offered. When cholesterol clefts are seen in biopsy specimens of necrobiosis, necrobiotic xanthogranuloma must be ruled out. In addition, when found in necrobiosis lipoidica diabeticorum, these clefts may imply diabetes mellitus with complications.
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4/9. necrobiotic xanthogranuloma: a report of four cases.

    Four patients are described who demonstrate the range of clinical and pathological features seen in necrobiotic xanthogranuloma with paraproteinaemia (NXG). Each patient had the typical periorbital yellow plaques with numerous well demarcated yellow indurated plaques on the trunk. All four patients had evidence of paraproteinaemia. The histopathology showed diagnostic features of intense necrobiosis with xanthomatization, including touton giant cells and the bizarre angulated giant cells of NXG. Previously, the patients had been diagnosed as atypical necrobiosis lipoidica, and the distinction between NXG and other necrobiotic conditions is discussed.
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5/9. Annular elastolytic giant cell granuloma.

    Annular elastolytic giant cell granuloma (AEGCG) is a clinical and histological entity, which has been described previously in various different terms. knowledge of AEGCG is of importance, since its misdiagnosis may lead to improper therapy; e.g. longterm tuberculostatic or corticosteroid treatment. In the present paper two patients with typical AEGCG are described and the clinical and histological differential diagnosis is discussed in detail.
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6/9. Atypical necrobiosis lipoidica of the face.

    A 36-year-old healthy man with atypical necrobiosis lipoidica of the face is described. The lesions were annular with a raised erythematous palpable border. The centres were slightly depigmented and atrophic, without telangiectasia or hair loss. Histopathological changes revealed prominent giant cells in small groups without clear granuloma formation. They were located at all levels of the drmis between the collagen bundles.
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7/9. A case of necrobiosis lipoidica treated with systemic corticosteroid.

    A 43-year-old woman had asymptomatic eruptions on the anterior sides of both legs for 5 years. biopsy specimens revealed the typical findings of necrobiosis lipoidica. An immunofluorescence study demonstrated no deposits of immunoglobulins, complement, or fibrinogen in vessel walls. Immunohistochemical staining revealed CD4 positive lymphocytes dominating in the lesion and strong expression of ICAM-1 on the endothelial cells, giant cells, lymphocytes and dermal fibroblasts in the lesion. The eruptions resolved remarkably with oral corticosteroid therapy.
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8/9. An unusual case of giant dermatofibroma in a patient with diabetes mellitus and necrobiosis lipoidica.

    A case of nine dermatofibromata, including a giant one, associated with necrobiosis lipoidica and diabetes mellitus type II is reported. This case is unusual because of the number and size of the tumors and their association with the above-mentioned pathologic conditions.
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9/9. necrobiosis lipoidica-like skin lesions in systemic sarcoidosis.

    A 62-year-old woman with systemic sarcoidosis developed erythematous plaques on her lower legs. Clinically, two kinds of skin lesions were distinguished; one type formed brownish-red plaques with induration suggesting plaque-type skin sarcoid, and the other formed purplish erythematous plaques with atrophic centers resembling necrobiosis lipoidica. In spite of this clinical appearance, a biopsy specimen from one of the latter lesions revealed typical skin sarcoid histology composed of discrete non-caseating granulomas, while that from one of the other lesions showed necrobiotic changes of collagen bundles surrounded by epitheloid histiocytes and foreign-body giant cells. Because cutaneous involvement of sarcoidosis may mimic necrobiosis lipoidica clinically and/or histologically, we diagnosed her skin lesions as necrobiosis-like skin sarcoid.
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