Combination therapy for Sneddon syndrome to reduce the incidence of cerebrovascular complications.

Autor: Narwutsch A; Department of Dermatology and Venereology, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany., Wohlrab J; Department of Dermatology and Venereology, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany., Sperfeld AD; Department of Neurology, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany.; Saxon Hospital Altscherbitz, Hospital for Psychiatry and Neurology, Schkeuditz, Germany., Sunderkötter C; Department of Dermatology and Venereology, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany.
Jazyk: angličtina
Zdroj: Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG [J Dtsch Dermatol Ges] 2024 Jul; Vol. 22 (7), pp. 947-954. Date of Electronic Publication: 2024 Jun 21.
DOI: 10.1111/ddg.15422
Abstrakt: Background: Sneddon syndrome is an occlusive vasculopathy that presents clinically with generalized livedo racemosa on the skin and transient ischemic attacks, strokes, and cognitive or motor deficits in the central nervous system. Antiplatelet or anticoagulant therapy is recommended. Due to the limited therapeutic efficacy and the resulting serious complications, we propose combination therapy with additional infusion cycles of alprostadil and captopril and report initial long-term results.
Patients and Methods: We performed a systematic retrospective analysis of all patients with primary Sneddon syndrome who received combination therapy in our clinic between 1995 and 2020. Therapeutic outcomes were evaluated using descriptive statistics compared to historical controls receiving monotherapy. We also analyzed the event rate of complications when combination therapy was discontinued.
Results: During the 99.7 patient-years of follow-up, there were no transient ischemic attacks and the stroke rate dropped to 0.02 per patient-year. In comparison, the rates of transient ischemic attacks and strokes in the historical controls ranged from 0.08 to 0.035 per patient-year. After discontinuation of alprostadil therapy, eight events occurred in three patients.
Conclusions: Combination therapy reduces the long-term incidence of ischemic events in patients with primary Sneddon syndrome.
(© 2024 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by Wiley‐VCH GmbH on behalf of Deutsche Dermatologische Gesellschaft.)
Databáze: MEDLINE
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