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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.
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.

Sneddon\'s syndrome is a rare condition characterized by the presence of neurocutaneous lesions, including reticular livedo and an increase in thrombotic risk, and it is associated with a greater risk of cerebrovascular disease. It is diagnosed through a skin biopsy and neurological manifestations. In this study, we present a clinical report of a 39-year-old nurse exposed to shift and night work in an intensive care unit. She was diagnosed with Sneddon\'s syndrome, whose workplace had to be readapted, considering the complications and restrictions of her condition and aiming her protection and healthcare promotion. Night work can exacerbate cerebral and cardiovascular events due to its impact on metabolism, blood pressure profile, and hormone concentrations. Therefore, given a syndrome characterized by an increased thrombotic risk associated with cerebrovascular events, the restriction of night work is imperative. The occupational physician is responsible for intervening in the workplace and preventing long-term consequences for employees.

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Sneddon syndrome, also known as livedo reticularis with cerebrovascular accidents, is a rare but chronic condition that affects blood vessels in the skin and brain. This syndrome is characterized by a net-like appearance on the skin, known as livedo reticularis, which occurs due to the constriction of blood vessels. In addition to skin manifestations, Sneddon syndrome is often associated with repeated neurological events, such as strokes or transient ischemic attacks. These neurological symptoms can vary in severity and can lead to various complications. Upon admission to the stroke unit, a 28-year-old female was found to have bilateral livedo reticularis affecting the soles and the dorsal sides of the hands. Patient evaluation is done through medical history, physical examination, routine laboratory tests, and other diagnostic procedures.

Sneddon syndrome may present with neurological findings such as transient ischemic stroke, strokes, seizures and/or headaches. However, a purplish, spider web-like skin finding called livedo reticularis may accompany the skin and precede neurological findings. Sneddon syndrome often affects women. Since it is vasculopathy affecting small and medium vessels, other organ findings may accompany. We present a 44-year-old Sneddon syndrome patient with monoparesis in her left lower extremity, livedo reticularis on her back and legs, and hypertension.

Sneddon\'s syndrome (SS) manifests through multiple strokes and livedo reticularis. Livedoid vasculopathy (VL) is characterized by a long history of foot and leg ulceration and histopathology indicating a thrombotic process. Arterial retinal branch occlusion is described in a 52-year-old male with VL. He did not present noticeable laboratory abnormalities, such as antiphospholipid antibodies, or a history of strokes. Retinal artery occlusion accompanied by VL could be a variant of Sneddon\'s syndrome. Optical coherence tomography angiography revealed a reduction in the macula\'s vascular layers in the asymptomatic eye, indicating localized microvascular changes as an evolving marker in the pathogenesis of SS.

This clinical case report aims to highlight the unusual presentation of Sneddon syndrome with a possible association with paroxysmal hemicrania. A medical record review was performed at a tertiary hospital in Riyadh, Saudi Arabia. Data collected include clinical evaluations and laboratory and imaging results. Informed consent was obtained. Hereby, we present a 27-year-old female who presented with multiple stroke attacks, along with severe headaches involving right retro-orbital pain with an eight-year history of spotted skin lesions. Initial unenhanced computed tomography (UCT) brain in the emergency showed left insular cortex hypodensity, revealing acute ischemic insult. Subsequent magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed acute ischemic infarct in the territory of the left middle cerebral artery (MCA) involving the insula and frontoparietal lobe. Further investigations were done, including cerebrospinal fluid (CSF) analysis and autoimmune and infectious workup, which were unrevealing. Skin biopsy of the lesions showed subcutaneous fat necrosis with nonspecific scattered fibrinogen positivity and was labeled as livedo reticularis vs. livedo racemosa. A Sneddon syndrome diagnosis can be very challenging, needing a high index of suspicion to direct the diagnostic investigations. Moreover, the presence of a severe headache is an unusual phenomenon that needs further study.

UNASSIGNED: The diagnosis of Sneddon Syndrome should be considered in adults with young-onset dementia accompanied by neuropsychiatric signs and livedo racemosa. Magnetic resonance imaging and cerebral angiography are essential. A cutaneous biopsy may help in the diagnosis.
UNASSIGNED: Sneddon syndrome (SS) is a clinical entity corresponding to a noninflammatory thrombotic vasculopathy that typically includes livedo racemosa and cerebrovascular ischemia. Psychiatric symptoms with cognitive impairment often occur but are rarely the inaugural symptoms. We present a case of secondary SS in a 45-year-old man in whom dementia and psychosis revealed the disease.

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