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[与抗心磷脂抗体相关的类脂质渐进性坏死(白色萎缩)]

[Livedoid vasculopathy (white atrophy) associated with anticardiolipin antibodies].

作者信息

Grasland A, Crickx B, Blanc M, Pouchot J, Vinceneux P

机构信息

Service de Médecine Interne V, Hôpital Louis-Mourier, 178, rue des Renouillers, 92700 Colombes.

出版信息

Ann Med Interne (Paris). 2000 Sep;151(5):408-10.

Abstract

BACKGROUND

Livedoid vasculopathy is an uncommon condition that affects young patients. It must be clinically distinguished from cutaneous vasculitis.

CASE REPORT

A 27-year-old man presented pain and skin eruptions involving the lower limbs, hands and trunk. The skin eruption predominated on the feet with purpuric lesions producing a livedoid pattern, ulcerations on both ankles and white atrophic scars on the dorsum of the feet. Skin biopsy showed dermic vessel thrombosis without leukocytoclasia. These features favored livedoid vasculopathy rather than vasculitis. Anticardiolipin antibodies were positive. The patient was started on antithrombotic therapy. The skin ulcerations regressed and no recurrence has been observed at 7-months follow-up.

DISCUSSION

Livedoid vasculopathy is characterized by painful purpuric lesions that generally occur on the lower limbs, and frequently ulcerate and heal leaving porcelain white atrophic scars (atrophic blanche). The histology evidences a thrombotic process. Livedoid vasculopathy is normally described as occurring as a sole entity, however thera have been reports of an association with anticardiolipin antibodies. This suggest a possible link with antiphospholipid syndrome.

摘要

背景

萎缩性血管病是一种影响年轻患者的罕见病症。必须在临床上将其与皮肤血管炎区分开来。

病例报告

一名27岁男性出现累及下肢、手部和躯干的疼痛及皮肤疹。皮疹主要出现在足部,有紫癜性损害形成萎缩性图案,双踝有溃疡,足背有白色萎缩性瘢痕。皮肤活检显示真皮血管血栓形成,无白细胞破碎现象。这些特征支持萎缩性血管病而非血管炎。抗心磷脂抗体呈阳性。患者开始接受抗血栓治疗。皮肤溃疡消退,在7个月的随访中未观察到复发。

讨论

萎缩性血管病的特征为疼痛性紫癜性损害,通常发生在下肢,且常发生溃疡,愈合后留下瓷白色萎缩性瘢痕(白色萎缩)。组织学显示为血栓形成过程。萎缩性血管病通常被描述为一种独立的病症,然而,有报道称其与抗心磷脂抗体有关。这提示与抗磷脂综合征可能存在联系。

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