A rare angiopathy with lesions of the skin and central nervous system: Sneddon syndrome and disease

Abstract

Sneddon syndrome is a rare, progressive disease that affects relatively young people, mainly female, and manifests itself in two main symptoms: livedo reticularis and recurrent cerebral infarctions. First described in 1965 by the English dermatologist Ian Bruce Sneddon, the syndrome named in his honor unites a heterogeneous group of diseases with different pathogenesis and similar clinical manifestations. Skin manifestations of the disease in the form of reticular livedo are benign, cerebrovascular manifestations are reduced to repeated cerebral infarctions, progressive dementia and can lead to the death of patients. The literature review describes the basic concepts of the etiology, pathogenesis of primary and secondary forms of Sneddon syndrome, and proposes the concept of Sneddon’s disease to describe the primary forms of the syndrome. The article contains photographs of patients from the clinical practice of the authors of the article. The review also describes methods of diagnosis and differential diagnosis of the disease and recommended methods of treatment, which is based on the elimination of additional risk factors for vascular damage and thrombosis, blood pressure control, lipid and carbohydrate metabolism, as well as the appointment of anticoagulant and disaggregant therapy. The use of anti-inflammatory or immunosuppressive therapy remains controversial. The main goal of treatment is the prevention of organic brain damage and neuropsychiatric complications. Unfortunately, the neuropsychiatric prognosis of Sneddon syndrome is relatively poor with the development of impaired memory, concentration, and visual-spatial skills. In exceptional cases, the progression of the disease can lead to death.