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硬斑病硬化斑块导致的淋巴管受压:儿童淋巴水肿的一种原发病因

Lymphatic compression by sclerotic patches of morphea: an original mechanism of lymphedema in a child.

作者信息

Samimi Mahtab, Maruani Annabel, Machet Marie-Christine, Baulieu Françoise, Machet Laurent, Lorette Gérard

机构信息

University François Rabelais, Tours, France.

出版信息

Pediatr Dermatol. 2010 Jan-Feb;27(1):58-61. doi: 10.1111/j.1525-1470.2009.01049.x.

Abstract

Lymphedema in children is mostly primary, due to lymphatic hypoplasia. Secondary lymphedema is caused by lymphatic injury or obstruction. We report the case of a child that developed a lymphedema of the left upper and lower extremities, with a simultaneous onset of ipsilateral hemicorporal morphea. We concluded that lymphatic obstruction was due to sclerosis from morphea. This is a unique, rarely reported mechanism of lymphedema. Lymphoscintigraphy revealed attenuated lymphatic flow in the left upper and lower limbs. Systemic corticosteroids were associated with slow improvement in the sclerotic patches. We simultaneously noticed an improvement in the lymphedema of limbs. Repeat lymphoscintigraphy revealed dramatically improved lymphatic function. This case suggests that at least in some cases lymphedema may be caused by morphea.

摘要

儿童淋巴水肿大多为原发性,由淋巴管发育不全引起。继发性淋巴水肿由淋巴管损伤或阻塞所致。我们报告一例儿童,其左上肢和下肢出现淋巴水肿,同时同侧半身硬斑病发作。我们得出结论,淋巴阻塞是由硬斑病导致的硬化引起的。这是一种独特的、鲜有报道的淋巴水肿机制。淋巴闪烁造影显示左上肢和下肢淋巴引流减弱。全身使用皮质类固醇使硬化斑块缓慢改善。我们同时注意到肢体淋巴水肿有所改善。重复淋巴闪烁造影显示淋巴功能显著改善。该病例表明,至少在某些情况下,淋巴水肿可能由硬斑病引起。

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