Neuromyelitis optica (NMO) is an autoimmune, relapsing central nervous system demyelinating disease. There is a known association between NMO and autoimmune disease. However, cutaneous findings in these cases and in the setting of isolated NMO have rarely been described. We report the case of a 60-year-old Chinese female who presented with nonspecific cutaneous findings and acute onset paraplegia and was subsequently found to be seropositive for aquaporin-4 (NMO IgG) antibodies, consistent with a diagnosis of NMO spectrum disorder. Serologic testing prompted by history and cutaneous stigmata revealed additional humoral derangements, together suggestive of an overlap syndrome with features of amyopathic dermatomyositis, rheumatoid arthritis and lupus erythematosus. We review the existing literature on the cutaneous manifestations of this entity. Awareness of the cutaneous signs and heralding symptoms of this devastating neurologic syndrome by dermatologists will ensure prompt diagnosis and initiation of treatment that can minimize neurologic sequelae.

Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG: The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805-815.
Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF, Lennon VA: Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006;59:566-569.
Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, Nakashima I, Weinshenker BG: A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004;364:2106-2112.
Wingerchuk DM, Hogancamp WF, O'Brien PC, Weinshenker BG: The clinical course of neuromyelitis optica (Devic's syndrome). Neurology 1999;53:1107-1114.
Pittock SJ, Lennon VA, de Seze J, Vermersch P, Homburger HA, Wingerchuk DM, Lucchinetti CF, Zéphir H, Moder K, Weinshenker BG: Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol 2008;65: 78-83.
Voss EV, Stangel M: Nervous system involvement of connective tissue disease: mechanisms and diagnostic approach. Curr Opin Neurol 2012;25:306-315.
Hara-Chikuma M, Verkman AS: Roles of aquaporin-3 in the epidermis. J Invest Dermatol 2008;128:2145-2151.
Cooper N, Te Water Naude J, Connor PP: Neuromyelitis optica complicating autoimmune lymphoproliferative syndrome in a 4-year-old girl. Br J Haematol 2011;152:247.
Pittock SJ, Lennon VA: Aquaporin-4 autoantibodies in a paraneoplastic context. Arch Neurol 2008;65:629-632.
Ontaneda D, Fox RJ: Is neuromyelitis optica with advanced age of onset a paraneoplastic disorder? Int J Neurosci 2014;124:509-511.
Suber TL, Casciola-Rosen L, Rosen A: Mechanisms of disease: autoantigens as clues to the pathogenesis of myositis. Nat Clin Pract Rheumatol 2008;4:201-209.
Levine SM: Cancer and myositis: new insights into an old association. Curr Opin Rheumatol 2006;18:620-624.
Casciola-Rosen L, Nagaraju K, Plotz P, Wang K, Levine S, Gabrielson E, Corse A, Rosen A: Enhanced autoantigen expression in regenerating muscle cells in idiopathic inflammatory myopathy. J Exp Med 2005;201:591-601.
Burd CJ, Kinyamu HK, Miller FW, Archer TK: UV radiation regulates Mi-2 through protein translation and stability. J Biol Chem 2008;283:34976-34982.
Karim S, Majithia V: Devic's syndrome as initial presentation of systemic lupus erythematosus. Am J Med Sci 2009;338:245-247.
Iwanaga Y, Hayashi S, Kawamura N, Ohyagi Y, Kira J: A case of neuromyelitis optica spectrum disorder associated with a limited cutaneous systemic sclerosis and Sjögren syndrome. Rinsho Shinkeigaku 2013;53:695-700.
Parperis K: A rare case of mixed connective tissue disease complicated with neuromyelitis optica. J Clin Rheumatol 2012;18:261-262.
Franciotta D, Zardini E, Caporali R, Piccolo L, Alberici E, Romani A, Bergamaschi R, Marchioni E, Ceroni M, Piccolo G: Systemic sclerosis in aquaporin-4 antibody-positive longitudinally extensive transverse myelitis. J Neurol Sci 2011;303:139-141.
Paira S, Benegas M, Ortiz A, Uña C, Rillo O, Mannucci P, Allievi A: Association of neuromyelitis optica (NMO) with autoimmune disorders: report of two cases and review of the literature. Clin Rheumatol 2010;29:1335-1338.
Jacobi C, Stingele K, Kretz R, Hartmann M, Storch-Hagenlocher B, Breitbart A, Wildemann B: Neuromyelitis optica (Devic's syndrome) as first manifestation of systemic lupus erythematosus. Lupus 2006;15:107-109.
Sekiguchi T, Ishibashi S, Kubodera T, Fukabori J, Uezato A, Kanbayashi T, Takahashi T, Yokota T, Mizusawa H: Anhidrosis associated with hypothalamic lesions related to anti-aquaporin 4 autoantibody. J Neurol 2011;258:2293-2295.
Elsone L, Townsend T, Mutch K, Das K, Boggild M, Nurmikko T, Jacob A: Neuropathic pruritus (itch) in neuromyelitis optica. Mult Scler 2013;19:475-479.
Nishiyama S, Ito T, Misu T, Takahashi T, Kikuchi A, Suzuki N, Jin K, Aoki M, Fujihara K, Itoyama Y: A case of NMO seropositive for aquaporin-4 antibody more than 10 years before onset. Neurology 2009;72:1960-1961.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.