Einstein (São Paulo) (Jun 2012)
Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome
Abstract
The Sneddon’s syndrome is a rare disorder characterized by theoccurrence of cerebrovascular disease associated with livedoreticularis. The antiphospholipid syndrome is the most frequent typeof acquired thrombophilia, defined by the occurrence of thrombosisor pregnancy morbidity in the presence of persistently positiveantiphospholipid antibodies. Approximately 80% of Sneddon’ssyndrome patients have an antiphospholipid antibody marker. Theseantibodies may play a pathogenetic role in some cases of Sneddon’ssyndrome, and many authors consider these two syndromes as thesame entity. Although clinical features of antiphospholipid syndromeand Sneddon’s syndrome may overlap, there is a distinction betweenclinical and laboratory evidence suggesting that these two entitiesare different diseases. A recent finding of coagulopathies, includingelevated levels of coagulation factor VII, decreased levels ofprotein S, and activated protein C in Sneddon’s syndrome patientssuggested a possible biological link between the vasculopathy anda primary coagulopathy. Moreover, the clinical course seems to beprogressive in Sneddon’s syndrome patients and includes increaseof disability and cognitive deterioration, more arterial involvement,and the antiphospholipid syndrome shows a more benign course.Both syndromes share clinical and laboratory features, and whetherSneddon’s syndrome represents a spectrum of antiphospholipidsyndrome remains unclear. Sneddon’s syndrome patients have aworse prognosis and may represent a subgroup of patients whodemands more rigorous follow-up. It is important to recognize theSneddon’s syndrome, particularly because stroke episodes may beprevented through appropriate treatment.
