title: Transverse myelitis associated with an itchy rash and hyperckemia: neuromyelitis optica associated with dermatitis herpetiformis.
abstract: OBJECTIVE
  Neuromyelitis optica is associated with severe neurodisability if not recognized and treated promptly. Several autoimmune disorders are associated with this condition and may vary in their presentation. It is essential that clinicians are aware of the uncommon presenting features of neuromyelitis optica and associated autoimmune conditions.
  
  
  METHODS
  A 53-year-old woman presented with nausea and vomiting and was noted to have an asymptomatic elevated creatinine kinase level, which improved with conservative management. She had a history of iron-deficiency anemia due to long-standing celiac disease that was managed with a gluten-free diet. She then presented with recurrent transverse myelitis and a vesicobullous rash over her arms and feet that was pruritic and excoriating. Skin biopsy results confirmed a clinical diagnosis of dermatitis herpetiformis and antibody test findings against aquaporin-4 were positive, leading to a diagnosis of neuromyelitis optica spectrum disorder. She was treated with methylprednisolone sodium succinate, plasma exchange, and azathioprine and has remained in remission.
  
  
  CONCLUSIONS
  This report highlights the association of neuromyelitis optica with dermatitis herpetiformis, which can present even without clinical features of celiac disease. Nausea, vomiting, and asymptomatic hyperCKemia should be recognized as rare presenting features of neuromyelitis optica.
authors.affiliation: Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, England2Alder Hey Children's NHS Foundation Trust, Liverpool, England.
authors.forename: Anand
authors.initials: A
authors.lastname: Iyer
authors.affiliation: Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, England.
authors.forename: Devipriya
authors.initials: D
authors.lastname: Rathnasabapathi
authors.affiliation: Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, England.
authors.forename: Liene
authors.initials: L
authors.lastname: Elsone
authors.affiliation: Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, England.
authors.forename: Kerry
authors.initials: K
authors.lastname: Mutch
authors.affiliation: Department of Histopathology, Aintree University Hospitals NHS Foundation Trust, Liverpool, England.
authors.forename: Monica
authors.initials: M
authors.lastname: Terlizzo
authors.affiliation: Department of Neurology, Lancashire Teaching Hospitals NHS Foundation Trust, Lancashire, England.
authors.forename: David
authors.initials: D
authors.lastname: Footitt
authors.affiliation: Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, England.
authors.forename: Anu
authors.initials: A
authors.lastname: Jacob
mesh_terms: D003402:Creatine Kinase / Q000096:biosynthesis* / Q000097:blood; D003874:Dermatitis Herpetiformis / Q000150:complications / Q000175:diagnosis / Q000201:enzymology*; D003937:Diagnosis, Differential; D005076:Exanthema / Q000150:complications / Q000175:diagnosis / Q000201:enzymology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009188:Myelitis, Transverse / Q000150:complications / Q000175:diagnosis / Q000201:enzymology*; D009471:Neuromyelitis Optica / Q000150:complications / Q000175:diagnosis / Q000201:enzymology*; D011537:Pruritus / Q000150:complications / Q000175:diagnosis / Q000201:enzymology*
chemical_list: D003402:Creatine Kinase