关键词: Sneddon综合征; 皮肤网状青斑; 缺血性卒中

Abstract:

Objective To investigate the clinical features, diagnosis and treatment in patients with Sneddon syndrome (SS), aiming to improve the clinical doctors’ understanding of this syndrome and enhance the ability of diagnosis and treatment of SS. Methods A total of 2 cases who were admitted to neurological disease center of Beijing Tiantan hospital, Capital Medical University during Aug.1st, 2014 to Aug.1st, 2017 and diagnosed with SS were reviewed. Their clinical descriptions, laboratory examinations, neuroimaging, cognitive function evaluation, pathological characteristic and treatment were analyzed. Results Clinical manifestations: 2 cases both suffered from generalized livedo racemosa of the skin and multiple recurrent ischemic stroke events. No specific blood biochemical and cerebral spinal fluid (CSF) abnormalities were found in both patients. Intracranial multiple abnormal signals was seen in magnetic resonance imaging (MRI). Intracranial and extracranial large vascular imaging evaluations were not significantly abnormal. Both patients had cognitive dysfunction. Skin biopsy was performed in 2 patients and the pathologic results were consistent with SS feature. Both cases were given antiplatelet and improving cognitive function therapy. Conclusion The diagnosis of SS should be made considering the patient's clinical manifestations, laboratory tests, imaging findings and the skin biopsy. Characteristic manifestations of the skin and multiple recurrent ischemic stroke events are helpful to the diagnosis of the disease. When necessary, skin biopsy can be performed in order for make clear diagnosis.

Key words: Sneddon syndrome; Livedo racemosa of skin; Ischemic stroke