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手部青少年透明纤维瘤病的非典型表现

Atypical Presentation of Juvenile Hyaline Fibromatosis of Hands.

作者信息

Dukan Ruben, Petroni Gautier, Bruneval Patrick, Pannier Stephanie, Masmejean Emmanuel H

机构信息

Hand, Upper Limb, and Peripheral Nerve Surgery Service, Georges-Pompidou European Hospital, Paris, France.

Hand, Upper Limb, and Peripheral Nerve Surgery Service, Georges-Pompidou European Hospital, Paris, France.

出版信息

J Hand Surg Am. 2022 Feb;47(2):195.e1-195.e5. doi: 10.1016/j.jhsa.2020.12.013. Epub 2021 Mar 14.

DOI:10.1016/j.jhsa.2020.12.013
PMID:33726934
Abstract

Juvenile hyaline fibromatosis is a rare disorder characterized by an extracellular accumulation of hyaline deposit. In the extremities, lesions may remain quiescent or gradually increase in size, eventually resulting in skin ulceration. There is no curative treatment. Surgery may allow some recovery of function, but recurrence is possible. We report a case of juvenile hyaline fibromatosis in both hands of a 25-year-old man who required multiple surgical procedures to address problems with function, pain, and appearance.

摘要

青少年透明纤维瘤病是一种罕见的疾病,其特征是透明沉积物在细胞外积聚。在四肢,病变可能保持静止或逐渐增大,最终导致皮肤溃疡。目前尚无治愈性治疗方法。手术可能使功能有所恢复,但仍有可能复发。我们报告一例25岁男性双手患青少年透明纤维瘤病的病例,该患者需要多次手术来解决功能、疼痛和外观问题。

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