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嗜酸性筋膜炎/泛发性硬斑病重叠综合征

Eosinophilic fasciitis/generalized morphea overlap.

作者信息

Heidary Noushin, Cheung Wang, Wang Nadia, Kamino Hideko, Franks Andrew G

机构信息

Department of Dermatology, New York University, USA.

出版信息

Dermatol Online J. 2009 Aug 15;15(8):2.

Abstract

A 50-year-old woman presented with a three-month history of violaceous, non-tender, indurated plaques on the chest, abdomen, breasts, and proximal portions of the arms and legs. An incisional biopsy specimen showed changes consistent with a diagnosis of inflammatory morphea. Over the course of one year, the patient began to develop signs and symptoms suggestive of a diagnosis of eosinophilic fasciitis, which included the characteristic groove sign on the upper extremities. Although our patient did not exhibit peripheral or histopathologic evidence of eosinophilia, the diagnosis of eosinophilic fasciitis could still be made because the aforementioned phenomena are not required for diagnosis. Multitude treatment regimes have been reported in the literature as single case reports or small patient series. Our patient was maintained on methrotrexate, oral glucocorticoids, and etanercept with improvement of skin lesions and mobility.

摘要

一名50岁女性,胸部、腹部、乳房以及手臂和腿部近端出现紫红色、无压痛、硬结性斑块,病程3个月。切开活检标本显示的变化符合炎症性硬斑病的诊断。在一年的病程中,患者开始出现提示嗜酸性筋膜炎诊断的体征和症状,包括上肢典型的沟槽征。尽管我们的患者未表现出嗜酸性粒细胞增多的外周或组织病理学证据,但仍可作出嗜酸性筋膜炎的诊断,因为上述现象并非诊断所必需。文献中作为个案报告或小患者系列报道了多种治疗方案。我们的患者接受甲氨蝶呤、口服糖皮质激素和依那西普治疗,皮肤病变和活动能力有所改善。

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