Successful treatment of Sneddon Syndrome with high dose intravenous immunoglobulin

Sneddon Syndrome is an orphan disease characterized by the clinical presence of livedo racemosa and cerebrovascular ischaemia. Its pathophysiology remains inadequately understood and no validated treatment modality has been established. In this case report of a 37-year-old woman, we present high-dose intravenous immunoglobulin (IVIg) as a potential successful treatment option. The diagnosis was confirmed through a deep spindle biopsy, revealing the typical intima proliferation. Furthermore, we found a positive medical history concerning neurological symptoms. The commonly applied high-dose anticoagulation had previously not resulted in improvement of the ulcers or pain reduction. The initiated therapy with IVIg significantly alleviated the pain after a single cycle and led to the complete healing of the ulcers 12 weeks later. This case of a patient with subsequent long-term responsiveness to IVIg supports the approach as a promising therapy alternative.