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[双侧臀部硬化性脂肪肉芽肿伴钙化。1例临床病例报告及文献复习]

[Bilateral sclerosing lipogranuloma of the gluteal region with calcification. Report of a clinical case and review of the literature].

作者信息

Iannello S, Greco M, Cavaleri A, Camuto M, Fagone S, Belfiore F

机构信息

Cattedra di Medicina Interna, Università degli Studi, Catania.

出版信息

Minerva Med. 2000 Mar-Apr;91(3-4):73-82.

Abstract

A rare case of large bilateral sclerosing lipogranuloma with multiple calcifications of gluteal region is described in an old female patient affected by a cerebrovascular disease. The lesions appeared as firm, nontender, plaques, 9-10 cm in diameter, covered with hyperpigmented skin. This uncommon disorder is discussed on the basis of data obtained from an extensive literature review. The term "sclerosing lipogranuloma" was coined in 1950, and it defines a disease of the subcutaneous fat, which for a trauma or unknown reasons undergoes necrosis of fat cells with the release of fat droplets into intercellular spaces and a peculiar local sclerosing granulomatous reaction of fatty tissue. The cytosteatonecrosis and sclerosing lipogranuloma, post-traumatic or secondary to injection of exogenous oily substances, usually localized in the breast of women and in genitalia of men, are relatively well known. Sclerosing lipogranulomatosis of the orbita and eyelides, an infrequent but severe complication after endonasal surgery, has also been reported. Rarely, the lipogranuloma can be spontaneous or idiopathic or primitive. A particular form of genetic diffuse lipogranulomatosis is the Farber's syndrome, firstly described on 1947. In our patient, the absence of trauma seems to indicate a primitive lipogranuloma. The presence of an acute rheumatic syndrome responsive to corticosteroids, and the positivity of antimitochondrial autoantibodies are in accordance with the report of sclerosing multiple lipogranulomatosis associated with a lupus-like syndrome. Because of the long duration and the absence of acute local symptoms, this syndrome can be considered benign with favorable prognosis, but the physician should know it.

摘要

本文描述了一例罕见的双侧臀部大型硬化性脂肪肉芽肿病例,该病例发生在一名患有脑血管疾病的老年女性患者身上。病变表现为质地坚硬、无压痛的斑块,直径9 - 10厘米,表面覆盖着色素沉着的皮肤。我们根据广泛文献综述获得的数据对这种罕见疾病进行了讨论。“硬化性脂肪肉芽肿”这一术语于1950年被提出,它定义了一种皮下脂肪疾病,这种疾病由于创伤或不明原因导致脂肪细胞坏死,脂肪滴释放到细胞间隙,并引发脂肪组织特有的局部硬化性肉芽肿反应。创伤后或继发于外源性油性物质注射的细胞脂肪坏死和硬化性脂肪肉芽肿,通常发生在女性乳房和男性生殖器部位,相对较为常见。眼眶和眼睑的硬化性脂肪肉芽肿病是鼻内手术后一种罕见但严重的并发症,也有相关报道。罕见情况下,脂肪肉芽肿可能是自发的、特发性的或原发性的。一种特殊形式的遗传性弥漫性脂肪肉芽肿病是法伯综合征,于1947年首次被描述。在我们的患者中,无创伤史似乎表明是原发性脂肪肉芽肿。对皮质类固醇有反应的急性风湿综合征的存在以及抗线粒体自身抗体呈阳性,与伴有狼疮样综合征的多发性硬化性脂肪肉芽肿的报道一致。由于病程较长且无急性局部症状,这种综合征可被认为是良性的,预后良好,但医生应该了解它。

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