Burg Maria Rosa, Mitschang Carolin, Goerge Tobias, Schneider Stefan Werner
Department of Dermatology and Venereology, University Medical Center Hamburg Eppendorf, Hamburg, Germany.
Department of Dermatology, University Hospital of Muenster, Muenster, Germany.
Front Med (Lausanne). 2022 Oct 3;9:1012178. doi: 10.3389/fmed.2022.1012178. eCollection 2022.
Livedoid vasculopathy is a rare, chronic-recurrent occlusive disorder in the microcirculation of dermal vessels. The clinical appearance is characterized by , painful ulceration, located in the distal parts of the lower extremities, followed by healing as porcelain-white, atrophic scars, the so-called . Different conditions that can promote a hypercoagulable state, such as inherited and acquired thrombophilias, autoimmune connective-tissue diseases and neoplasms, can be associated with livedoid vasculopathy. Therefore, livedoid vasculopathy is currently considered to be a coagulation disorder, clearly distinguished from inflammatory vasculitis. Although there are hints to hypercoaguability and secondary inflammation, pathophysiology is not completely understood. Diagnosis is made by synopsis of history, clinical and histopathological findings. Early and adequate therapy is essential to maintain life quality and avoid irreversible complications. Better understanding of molecular mechanisms is required to establish appropriate therapy regimens. This article presents the current state of knowledge about livedoid vasculopathy and proposes an algorithmic approach for diagnosis and therapy.
萎缩性紫癜病是一种罕见的、慢性复发性真皮血管微循环闭塞性疾病。其临床表现特征为下肢远端出现疼痛性溃疡，随后愈合形成瓷白色萎缩性瘢痕，即所谓的……。多种可促进高凝状态的情况，如遗传性和获得性血栓形成倾向、自身免疫性结缔组织病及肿瘤，都可能与萎缩性紫癜病相关。因此，目前认为萎缩性紫癜病是一种凝血障碍性疾病，与炎症性血管炎有明显区别。尽管有高凝状态及继发性炎症的迹象，但其病理生理学尚未完全明确。诊断需综合病史、临床及组织病理学检查结果。早期且充分的治疗对于维持生活质量及避免不可逆并发症至关重要。需要更好地了解分子机制以制定合适的治疗方案。本文介绍了关于萎缩性紫癜病的当前知识状况，并提出了诊断和治疗的算法方法。
