Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Dermatologie et Allergologie, Hôpital Tenon, 75020 Paris, France.
Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Neurologie, Hôpital Saint-Antoine, 75012 Paris, France.
Ann Dermatol Venereol. 2022 Mar;149(1):3-13. doi: 10.1016/j.annder.2021.08.007. Epub 2021 Nov 2.
The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients.
自 1965 年以来，人们一直使用 Sneddon 综合征（SS）一词来描述一种血管病变，其特征是脑血管疾病与网状青斑并存。SS 可分为抗磷脂抗体阳性（aPL+）或抗磷脂抗体阴性（aPL-）。关于 aPL-SS 知之甚少；在这篇综述中，我们描述了 aPL-SS 的流行病学和发病机制，以及临床和组织学特征。我们根据神经和心脏受累情况讨论了最近的发现。此外，还讨论了可能同时出现网状青斑和中风的疾病的鉴别诊断。最后，我们讨论了实际生活中的实际问题，例如要进行的初始检查、长期随访以及 aPL-SS 患者的治疗管理。
