Antiphospholipid-negative Sneddon's syndrome: A comprehensive overview of a rare entity

F Assan; L Bottin; C Francès; P Moguelet; S Tavolaro; A Barbaud; D de Zuttere; S Alamowitch; F Chasset

doi:10.1016/j.annder.2021.08.007

Antiphospholipid-negative Sneddon's syndrome: A comprehensive overview of a rare entity

Ann Dermatol Venereol. 2022 Mar;149(1):3-13. doi: 10.1016/j.annder.2021.08.007. Epub 2021 Nov 2.

Authors

F Assan¹, L Bottin², C Francès¹, P Moguelet³, S Tavolaro⁴, A Barbaud¹, D de Zuttere⁵, S Alamowitch², F Chasset⁶

Affiliations

¹ Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Dermatologie et Allergologie, Hôpital Tenon, 75020 Paris, France.
² Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Neurologie, Hôpital Saint-Antoine, 75012 Paris, France.
³ Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Pathologie, Hôpital Tenon, 75020 Paris, France.
⁴ Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Radiologie, Hôpital Tenon, 75020 Paris, France.
⁵ Service d'Explorations Fonctionnelles, Hôpital Franco-Britannique, 92300 Levallois-Perret, France.
⁶ Sorbonne University, Faculté de Médecine Sorbonne Université, AP-HP, Service de Dermatologie et Allergologie, Hôpital Tenon, 75020 Paris, France. Electronic address: francois.chasset@aphp.fr.

PMID: 34740467
DOI: 10.1016/j.annder.2021.08.007

Abstract

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.

Keywords: Antiphospholipid; Cerebral infarction; Livedo racemosa; Sneddon's syndrome.

Publication types

Review

MeSH terms

Antiphospholipid Syndrome* / complications
Antiphospholipid Syndrome* / diagnosis
Antiphospholipid Syndrome* / epidemiology
Diagnosis, Differential
Humans
Livedo Reticularis* / diagnosis
Livedo Reticularis* / epidemiology
Livedo Reticularis* / etiology
Sneddon Syndrome* / complications
Sneddon Syndrome* / diagnosis
Sneddon Syndrome* / epidemiology