Department of Dermatology, University Hospital Münster, Germany.
J Dtsch Dermatol Ges. 2013 May;11(5):407-10. doi: 10.1111/ddg.12064. Epub 2013 Feb 26.
Livedoid vasculopathy is a rare, chronic, recurrent disease of the cutaneous microcirculation. Its typical clinical manifestation is a triad which consists of livedo racemosa of the skin, episodic painful ulcerations of the distal aspects of the legs and a healing process leaving small porcelain-white scars called atrophie blanche. As an important result of recent research, livedoid vasculopathy has been defined as a coagulation disorder classified as a vasculopathy different from inflammatory vasculitis. This differentiation adds to the current pathophysiologic understanding and supports the therapeutic rationale with respect to the use of new systemic anticoagulants. The prevention of irreversible residual scarring and the improvement of patientsí quality of life are the main goals in treating cutaneous infarction and require early and consequent treatment. This article presents current knowledge on diagnosing this rare disease and offers practical guidance on its therapy.
皮肤白细胞破碎性血管炎是一种罕见的、慢性的、复发性皮肤微循环疾病。其典型临床表现为三联征，包括皮肤匐行性青斑、小腿远端间歇性疼痛性溃疡和愈合过程中遗留的小瓷白色瘢痕（萎缩性白色瘢痕）。作为最近研究的重要结果，皮肤白细胞破碎性血管炎已被定义为一种凝血障碍，归类为不同于炎症性血管炎的血管病。这种分化增加了目前对病理生理学的理解，并支持了使用新型全身性抗凝剂的治疗原理。预防不可逆转的残余瘢痕形成和改善患者的生活质量是治疗皮肤梗死的主要目标，需要早期和持续的治疗。本文介绍了诊断这种罕见疾病的最新知识，并提供了其实践治疗的指导。
