Susac syndrome with prominent dermatological findings and a prompt response to intravenous immunoglobulin, steroids, and rituximab: a case report

Gertner, Elie; Rosenbloom, Michael

doi:10.1186/s13256-016-0917-4

Cited by 18 publications

(17 citation statements)

References 14 publications

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“…Skin changes are reported in <5% of the cases. The presence of LRa may serve as a marker of endothelial cell involvement [59,60]. According to specific diagnostic criteria developed by the European Susac Consortium, the diagnosis can be made if a retinal, vestibulocochlear or brain involvement is documented [57].…”

Section: Susac Syndromementioning

confidence: 99%

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Mitri

Enk

Bersano

et al. 2020

Euro J of Neurology

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Livedo is a net‐like violaceous skin pattern. It can be classified as physiological or pathological. The physiological livedo reticularis usually appears in cold conditions, whereas the pathological and irregular livedo, which persists in warm temperatures, is often labeled as ‘livedo racemosa’. Some neurological pathologies are associated with livedo, most commonly those with an inflammatory component or those derived from systemic disorders. The present review summarizes the most important central and peripheral neurological diseases in pediatric and adult age groups associated with livedo, providing physicians with an overview of the clinical presentation, etiology, diagnosis and management of these conditions.

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Section: Susac Syndromementioning

confidence: 99%

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Mitri

Enk

Bersano

et al. 2020

Euro J of Neurology

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“…Se han descrito casos asociados a mialgias y/o artralgias. Además, lesiones dermatológicas como livedo reticularis descrito en un caso 9 , los autores lo relacionan a alteraciones del flujo sanguíneo en las arterias dermales.…”

Section: Discussionunclassified

“…Siempre debe sospecharse a pesar de que la tríada clínica no esté presente inicialmente porque el síndrome puede presentarse completo en semanas hasta incluso 2 años posterior al síntoma inicial 6 . La presentación clínica inicial con la tríada completa es infrecuente, por lo que el diagnóstico no se debe hacer solo en la presencia de la tríada 3,9 . La confirmación clínica de este síndrome se basa en la realización de diversos exámenes, como RM, angiografía fluorescente de la retina y audiometría entre otros 3 .…”

Section: Discussionunclassified

Síndrome de Susac. Caso clínico y revisión

et al. 2018

Rev. Otorrinolaringol. Cir. Cabeza Cuello

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(SS) es una enfermedad autoinmune microangiopática poco frecuente, caracterizada por la tríada clínica: encefalopatía, alteraciones visuales e hipoacusia. Es una entidad de prevalencia desconocida dado su difícil diagnóstico debido a que la mayoría de las veces no se presenta con la tríada clínica clásica, y, dado que presenta múltiples diagnósticos diferenciales. Se reporta un caso de SS presentado en el Hospital del Salvador, con descripción de sus manifestaciones clínicas, estudio y manejo inicial. Además de una revisión en la literatura sobre la variabilidad de los hallazgos clínicos y el manejo de esta patología.

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“…Hearing loss can be a dramatic and severely disabilitating feature of SS as it is often irreversible and may require cochlear implants or lifelong hearing devices (6). Other manifestations include myalgia, arthralgia and skin lesions (10). A broad spectrum of differential diagnosis includes autoimmune diseases (systemic lupus erythematosus, neuro-Behçet disease) demyelinating diseases (multiple sclerosis, acute disseminated encephalomyelitis), and vascular occlusion (atherosclerosis or embolic disorders).…”

Section: Case Reportmentioning

confidence: 99%

Headache in a young woman: do not forget Susac’s syndrome

et al. 2017

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SUMMARYSusac syndrome is a rare disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. This underdiagnosed condition needs to be considered in the differential diagnosis of a broad variety of disorders. An early diagnosis is important as treatment can halt disease progression and prevent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the importance of an aggressive therapeutic approach, including the combination of steroids and other cytotoxic drugs.Key words: Susac's syndrome; Headache; Young woman. Reumatismo, 2017; 69 (3): 122-125 n INTRODUCTION S usac's syndrome (SS), also known as retinocochleocerebral vasculopathy, is a rare condition that was first reported in 1973 (1). Although SS pathophysiology remains mysterious, it is now thought that it is most probably an immune-mediated endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear (2). Although the clinical triad of encephalopathy, branch retinal artery occlusions (BRAO), and sensorineural hearing loss, representing the main feature of SS, is increasingly recognized, SS remains often underdiagnosed particularly at the early stage (3). Herein, we report a case of a 30-year-old woman with SS and we highlight the difficulties of an early diagnosis and adequate treatment. n CASE REPORTA 31-year-old woman was admitted to our department with a history of two months of severe headache, blurry vision and sudden painless vision deterioration in the left eye (LE). A review of the remaining medical history was unremarkable. Particularly, there were no hearing difficulties, no cognitive impairment, no memory loss, no vertigo, no psychiatric disorders and no weakness. The patient denied any infectious exposure or seizure. On physical examination, she was afebrile, and conscious. Blood pressure was normal and all peripheral pulses were palpable and symmetrical. There were no pertinent findings especially from examination of cardiovascular, pulmonary and neurological systems. Ophthalmic examination in the LE revealed BRAO with retinal cloudy swelling at the upper part of the macula. The rest of the ophthalmic examination showed no abnormalities. The right eye (RE) was completely normal with vision of 10/10. Laboratory tests showed no relevant alterations in blood cell count, hemostasis tests, renal and liver function, electrolytes, or lipid panel. There was no biological inflammatory syndrome. Tests for antinuclear antibodies (ANA), anti double stranded DNA (dsDNA), extractable nuclear antigen (ENA) panel, anti-neutrophil cytoplasmic antibodies (ANCA), anti-prothrombin antibodies and antiphospholipid antibodies were negative. Electrocardiogram, and echography of the supra-aortic trunks showed no ab- o n l y vent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the...

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Susac syndrome with prominent dermatological findings and a prompt response to intravenous immunoglobulin, steroids, and rituximab: a case report

Cited by 18 publications

References 14 publications

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Livedo racemosa in neurological diseases: an update on the differential diagnoses

Síndrome de Susac. Caso clínico y revisión

Headache in a young woman: do not forget Susac’s syndrome

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