J. Berciano
Neurosciences and History 2026;14(1): 26-42
Type of article: REVIEW
AUTOR
J. Berciano
Professor emeritus ad honorem, University of Cantabria, Santander, Spain.
Former Head of the Service of Neurology, University Hospital Marqués de Valdecilla (IDIVAL), Santander, Spain.
CIBERNED, Santander, Spain.
ABSTRACT
Objective. Sneddon syndrome (SnS) is a rare entity characterized by livedo racemosa and cerebrovascular lesions. SnS is now regarded as common manifestation of different disease entities, including primary SnS, primary antiphospholipid syndrome–related SnS, and systemic lupus erythematosus–related SnS.
Development. The paper is divided into four parts. First, the differences between livedo reticularis and livedo racemosa are reviewed. Second, a historical analysis of the original descriptions of SnS is presented. Third, the results of the first prospective study of primary SnS, conducted by the author between 1977 and 1981 at Valdecilla Hospital, are set out. The series comprised eight patients (seven women and one man), representing 0.26% of the total number of cases of cerebrovascular disease admitted to the hospital during the five-year study period. Age at admission ranged from 18 to 59 years (mean, 46), whereas age of symptom onset varied between 10 and 33 years (mean, 19). The clinical, hereditary, neuroradiological and pathological features are reviewed. Finally, an updated review of the literature on SnS is presented, placing particular emphasis on nosological relationships of primary SnS with cerebral thromboangiitis obliterans and Divry–van Bogaert syndrome.
Conclusions. Primary SnS represents a new form of genetic, progressive, occlusive and non-inflammatory arteriopathy, which mainly involves medium-size vessels. Primary SnS, cerebral thromboangiitis obliterans, and Divry–van Bogaert syndrome should probably be considered as a single nosological entity.
KEYWORDS
Antiphospholipid syndrome, livedo racemosa, moyamoya syndrome, occlusive arteriopathy, Sneddon syndrome, stroke
SUPPLEMENTARY MATERIAL
Neurosciences and History 2026;14(1): 26-42