title: Susac syndrome with prominent dermatological findings and a prompt response to intravenous immunoglobulin, steroids, and rituximab: a case report.
abstract: BACKGROUND
  Susac syndrome (retinocochleocerebral vasculopathy) is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. It presents with encephalopathy, branch retinal artery occlusions, and hearing loss. The condition is often under recognized because the clinical symptoms may present at different times and physicians may be unfamiliar with the syndrome. Peripheral findings would be helpful in early diagnosis. There are numerous treatment regimens proposed with varying effectiveness.
  
  
  METHODS
  We report the case of a 22-year-old Caucasian man in whom there were prominent skin findings, including livedo reticularis and a micropapular eruption which responded promptly to treatment suggesting that skin involvement may facilitate earlier diagnosis. Rituximab has occasionally been used in more refractory disease. We observed a prompt response to the combination of intravenous immunoglobulin, corticosteroids, and rituximab instituted immediately after diagnosis.
  
  
  CONCLUSIONS
  A careful search for dermatological manifestations may help with earlier diagnosis. Skin findings may be another marker of endothelial cell involvement. Early use of rituximab as part of the therapeutic regimen may be warranted.
authors.affiliation: Section of Rheumatology, Regions Hospital, 640 Jackson Street, St. Paul, MN, 55101, USA. elie.x.gertner@healthpartners.com.
authors.forename: Elie
authors.initials: E
authors.lastname: Gertner
authors.affiliation: Section of Neurology, 401 Phalen Boulevard, Saint Paul, MN, 55130, USA.
authors.forename: Michael H
authors.initials: MH
authors.lastname: Rosenbloom
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging; D005654:Fundus Oculi; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D054068:Livedo Reticularis / Q000188:drug therapy* / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use*; D055955:Susac Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D000069283:Rituximab; D008775:Methylprednisolone
keywords: Autoimmune endotheliopathy; Livedo reticularis; Susac syndrome