ABSTRACT
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.
Subject(s)
Antiphospholipid Syndrome , Livedo Reticularis , Sneddon Syndrome , Stroke , Humans , Female , Adult , Sneddon Syndrome/complications , Sneddon Syndrome/diagnosis , Livedo Reticularis/complications , Stroke/etiology , Skin , Antibodies, Antiphospholipid , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosisABSTRACT
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.
Subject(s)
Retinal Artery Occlusion , Sneddon Syndrome , Humans , Male , Retinal Artery Occlusion/etiology , Retinal Artery Occlusion/diagnostic imaging , Middle Aged , Sneddon Syndrome/complications , Tomography, Optical Coherence , Livedo ReticularisSubject(s)
Sneddon Syndrome , Humans , Sneddon Syndrome/complications , Sneddon Syndrome/diagnosis , SkinABSTRACT
The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients.
Subject(s)
Antiphospholipid Syndrome , Livedo Reticularis , Sneddon Syndrome , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/epidemiology , Diagnosis, Differential , Humans , Livedo Reticularis/diagnosis , Livedo Reticularis/epidemiology , Livedo Reticularis/etiology , Sneddon Syndrome/complications , Sneddon Syndrome/diagnosis , Sneddon Syndrome/epidemiologyABSTRACT
No disponible
Subject(s)
Humans , Female , Middle Aged , Renal Insufficiency, Chronic/etiology , Sneddon Syndrome/complications , Albuminuria/diagnosis , Anticoagulants/therapeutic use , Hypertension/complications , Albuminuria/urine , Creatinine/blood , Antibodies, Antinuclear/analysis , Autoimmunity , Biopsy , Fluorescent Antibody TechniqueABSTRACT
BACKGROUND: The presence of livedo reticularis in patients with ischaemic stroke is associated with Sneddon syndrome (SS). Our objective was to present the clinical features of SS patients and to assess the role of antiphospholipid antibodies (APL). METHODS: Consecutive patients, diagnosed with SS between 1996 and 2017, were retrospectively reviewed for their demographic, neurological, dermatological, cardiac and extracerebral vascular features. Diagnosis of SS was made only if other causes of stroke were excluded. Patients with and without APL were included and compared for their clinical features. RESULTS: Fifty-three patients (79% female) were included, of whom 14 patients were APL-positive. Median age at diagnosis was 40 years. Approximately 60% of the patients had ≥ 3 cardiovascular risk factors. There were 129 previous vascular events (66 ischaemic strokes, 62 TIAs and 1 amaurosis fugax) during a median period of 2 years between the first event and diagnosis of SS. Skin biopsy was positive for SS in 29 patients (67%), mostly showing a thickened vessel wall with neovascularization in the deep dermis. After a median follow-up of 28 months, 4 patients, either on antiplatelet or oral anticoagulation therapy, had a recurrent stroke. There were few statistically significant differences between APL-negative and APL-positive patients, including the number of vascular events before diagnosis. CONCLUSIONS: SS predominantly affects young women with a relatively large number of cardiovascular risk factors. Clinical features of SS are comparable across different studies. We found no differences in the main clinical features between APL-positive and APL-negative patients.
Subject(s)
Antiphospholipid Syndrome , Brain Ischemia , Sneddon Syndrome , Stroke , Antibodies, Antiphospholipid , Brain Ischemia/complications , Brain Ischemia/epidemiology , Female , Humans , Male , Retrospective Studies , Sneddon Syndrome/complications , Sneddon Syndrome/diagnosis , Sneddon Syndrome/epidemiology , Stroke/complications , Stroke/epidemiology , Stroke/therapyABSTRACT
Herein, we report a case-based review of the Sneddon Syndrome (SS), a rare chronic condition which affects small to medium blood vessels. It is known by its skin presentation, livedo racemosa (LRC), and the relapsing cerebrovascular events. However, neither LRC nor cerebrovascular events are exclusive to SS. A 36-year-old female with history of mitral valve prolapse, hypothyroidism, Raynaud phenomenon, hypertension, migraines, and four episodes of transient ischemic attacks (TIA), presented to our clinic with new skin findings, suggestive of LRC. Based on her previous history, current presentation and skin biopsy results, she was diagnosed with SS secondary to antiphospholipid syndrome. The present report illustrates the difficulty in recognizing SS and how the heterogeneity of the disease may be contributing to the difficulty making a distinct diagnosis.
Subject(s)
Antiphospholipid Syndrome/diagnosis , Sneddon Syndrome/diagnosis , Adult , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/drug therapy , Antirheumatic Agents/administration & dosage , Factor Xa Inhibitors/administration & dosage , Female , Humans , Hydroxychloroquine/administration & dosage , Rivaroxaban/administration & dosage , Skin/pathology , Sneddon Syndrome/complications , Sneddon Syndrome/drug therapyABSTRACT
OBJECTIVE: Sneddon's syndrome is a cerebrocutaneous non-inflammatory progressive distal arteriopathy, characterized by livedo racemosa, stroke, and neuropsychiatric symptoms. Our aim was to highlight the characteristic neuroimaging features of Sneddon's syndrome that might be helpful to clinicians in timely diagnosis of this entity. METHODS: Twelve patients (median age 49 years, 11 female) with primary Sneddon's syndrome, diagnosed in last 10 years, were analyzed from the perspective of magnetic resonance imaging (MRI) features. In addition, a novel pseudoangiomatosis score was defined for grading angiographic abnormalities (range: 0 to 6). RESULTS: Median interval from the onset of neurological symptoms to diagnosis was 6 years. Presentation was with acute stroke in 5, seizures in 3, dementia/speech problems in 2, seizures plus cognitive dysfunction in 1, and chronic progressive hemiparesis in 1. All patients had a typical lesion pattern on MRI. This included multiple (median 3) cortical-subcortical supratentorial and cerebellar non-territorial infarcts, accompanied by multifocal cerebral atrophy. Of note, large territorial infarcts due to cerebral parent artery occlusion, an embolic pattern with multi-territorial involvement on diffusion-weighted imaging, small vessel disease features like severe white matter involvement or lacunar infarcts, and cerebral hemorrhage in the absence of anticoagulation were not observed. MRI lesion severity was not correlated with angiographic arteriopathy severity, clinical stage, or presentation symptoms. CONCLUSION: Sneddon's syndrome is characterized by highly typical clinico-radiological features. Brain MRI has diagnostic value. By knowing the characteristics of the syndrome, misdiagnosis and potentially harmful treatment can be prevented in this entity that might pose a diagnostic challenge.
Subject(s)
Cerebrovascular Disorders , Sneddon Syndrome , Brain/diagnostic imaging , Cerebral Hemorrhage , Cerebrovascular Disorders/complications , Cerebrovascular Disorders/diagnostic imaging , Female , Humans , Magnetic Resonance Imaging , Middle Aged , Sneddon Syndrome/complications , Sneddon Syndrome/diagnostic imagingABSTRACT
Sneddon's syndrome is a rare disease characterized by cerebrovascular events and livedo racemosa. There are often autoimmunological comorbidities, especially antiphospholipid antibody syndrome. The underlying pathophysiology is still not fully clarified. A causal therapy does not exist. The reported case shows a patient with a thrombophilic form of Sneddon's syndrome with the main symptoms of headache and thromboembolic events. Symptoms, laboratory parameters, histology and differential diagnoses are explained.
Subject(s)
Cerebrovascular Disorders/complications , Headache/complications , Livedo Reticularis/complications , Sneddon Syndrome/complications , Cerebrovascular Disorders/immunology , Diagnosis, Differential , Humans , Livedo Reticularis/immunology , Sneddon Syndrome/immunology , Thromboembolism/complicationsABSTRACT
Sneddon's syndrome is a rare, noninflammatory thrombotic vasculopathy characterized by the combination of livedo racemosa, recurrent stroke, and histopathological skin lesions of endarteritis obliterans. Although multiorgan involvement suggests its systemic nature, detailed pathological description of affected organs - including the kidney - is exceptional. We report a case of Sneddon's syndrome with chronic kidney disease, associated with features of endarteritis obliterans in the skin and the kidney. The clinical presentation of our patient is compared to previously reported cases of Sneddon's syndrome with biopsy-proven kidney disease. We also discuss the differential diagnosis, pathophysiological mechanisms, relationship with antiphospholipid syndrome, and management of patients with Sneddon's syndrome and kidney disease. This clinical observation supports the systemic nature of Sneddon's syndrome and provides insights into the mechanisms by which this rare but probably underdiagnosed disease alters kidney function.â©.
Subject(s)
Renal Insufficiency, Chronic/etiology , Sneddon Syndrome/complications , Female , Humans , Middle AgedABSTRACT
We present the case of a 58-year-old woman with multiple brain infarcts and livedo racemosa, a distinctive branched and irregular skin discoloration, on the trunk and limbs. Skin biopsy showed intimal proliferation with occlusion of a subcutaneous arteriole. We diagnosed Sneddon's syndrome, a rare neurocutaneous disorder likely caused by a noninflammatoryvasculopathy.
Subject(s)
Brain Infarction/etiology , Skin Diseases/etiology , Sneddon Syndrome/diagnosis , Biopsy , Brain/pathology , Brain Infarction/pathology , Female , Humans , Middle Aged , Skin/pathology , Skin Diseases/diagnosis , Sneddon Syndrome/complications , Vascular Diseases/etiologyABSTRACT
Sneddon syndrome (SS) is an episodic or chronic, slowly progressive disorder and characterized by generalized livedo racemosa (patchy, violaceous, skin discoloration) and recurrent cerebrovascular events. The histopathology of skin and brain is remarkable for a noninflammatory thrombotic vasculopathy involving medium- and small-sized dermal and cerebral arteries, respectively. Approximately 80% of the SS patients are women with a median age of diagnosis at 40 years. However, the onset of the disease during childhood have been reported. Etiopathogenesis of SS is unknown with 2 primary mechanisms proposed - autoimmune/inflammatory versus thrombophilia. SS is primarily classified as antiphospholipid positive or negative type. Neurological manifestations usually occur in 3 phases: (1) prodromal symptoms such as headaches, dizziness, and vertigo, (2) recurrent strokes, and (3) early onset dementia. Livedo racemosa precedes the onset of recurrent strokes by more than 10 years, but in many instances, the significance of the skin lesion is recognized only after the appearance of the stroke. The involvement of the heart valves, systolic labile hypertension, and retinal changes are also commonly associated with this syndrome. Treatment of SS is primarily based on anecdotal reports. Antiplatelet and antithrombotic agents are used for secondary stroke prophylaxis, and a recent study showed a relatively lower stroke recurrence rate with the universal use of antiplatelet/antithrombotic agents. Routine use of anti-inflammatory or immunosuppressive therapies is controversial. Neuropsychiatric prognosis of SS is relatively poor with predominant deficits in the concentration, attention, visual perception, and visuospatial skills.
Subject(s)
Cerebral Arteries/pathology , Livedo Reticularis/etiology , Skin/blood supply , Sneddon Syndrome/complications , Stroke/etiology , Anti-Inflammatory Agents/therapeutic use , Cerebral Arteries/drug effects , Fibrinolytic Agents/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Livedo Reticularis/pathology , Livedo Reticularis/physiopathology , Livedo Reticularis/prevention & control , Platelet Aggregation Inhibitors/therapeutic use , Recurrence , Risk Factors , Sneddon Syndrome/drug therapy , Sneddon Syndrome/pathology , Sneddon Syndrome/physiopathology , Stroke/pathology , Stroke/physiopathology , Stroke/prevention & control , Treatment OutcomeABSTRACT
In this case report, a 28-year-old woman known with slight aortic regurgitation presented with partial complex epileptic seizures. On examination, livedo reticularis was noted, and cerebral MRI scans showed signs of clinical silent old lacunar infarctions. She was persistently triple positive for antiphospholipid antibodies in high titres and fulfilled the antiphospholipid syndrome criteria. The patient was diagnosed with Sneddon's syndrome, which is a rare thrombotic vasculopathy characterised by the combination of cerebrovascular disease with livedo reticularis. Her seizures stopped, after anticoagulation therapy with warfarin was initiated.