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Short communication| Volume 319, ISSUE 1-2, P152-155, August 15, 2012

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Neuromyelitis optica in a patient with pemphigus foliaceus

      Abstract

      Neuromyelitis optica (NMO, also eponymously known as Devic's disease) is an immune‐mediated demyelinating disease of the central nervous system that can lead to significant disability. Pediatric NMO is a rare disorder often reported after an infection. The authors report a 16 year-old female patient with pemphigus foliaceus who developed subacute optic neuritis followed by cervical transverse myelitis. Restricted distribution of the lesions in the optic nerve and spinal cord was confirmed by ophthalmological evaluation and magnetic resonance imaging of the brain and spinal cord. She was started on intravenous methylprednisolone and then given a maintenance oral prednisone. Subsequently, she was treated with a nonsteroidal immunosuppressant, mycophenolate mofetil, with a target dose of 1000 mg twice a day. Over the course of months, patient noted significant recovery of previous deficits and resolution of the cervical cord enhancement, expansion and cystic dilatation that was previously seen. This case is noteworthy for being the first patient reported with neuromyelitis optica associated with pemphigus foliaceus.

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      References

        • Wingerchuk D.M.
        • Hogancamp W.F.
        • O'Brien P.C.
        • Weinshenker B.G.
        The clinical course of neuromyelitis optica (Devic's syndrome).
        Neurology. 1999; 53: 1107e14
        • Huppke P.
        • Bluthner M.
        • Bauer O.
        • Stark W.
        • Reinhardt K.
        • Huppke B.
        • et al.
        Neuromyelitis optica and NMO-IgG in European pediatric patients.
        Neurology. 2010; 75: 1740-1744
        • Karussis D.
        • Leker R.R.
        • Ashkenazi A.
        • Abramsky O.
        A subgroup of multiple sclerosis patients with anticardiolipin antibodies and unusual clinical manifestations: do they represent a new nosological entity?.
        Ann Neurol. 1998; 44: 629-634
        • Harada T.
        • Ohashi T.
        • Harada C.
        • Yoshida K.
        • Maguchi S.
        • Moriwaka F.
        • et al.
        A case of bilateral optic neuropathy and recurrent transverse myelopathy associated with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA).
        J Neuroophthalmol. 1997; 17: 254-256
        • Mok C.C.
        • Lau C.S.
        Transverse myelopathy complicating mixed connective tissue disease.
        Clin Neurol Neurosurg. 1995; 97: 259-260
        • Kister I.
        • Gulati S.
        • Boz C.
        • Bergamaschi R.
        • Piccolo G.
        • Oger J.
        • et al.
        Neuromyelitis optica in patients with myasthenia gravis who underwent thymectomy.
        Arch Neurol. 2006; 63: 851-856
        • Marques A.
        Da neuromieliteótica: contribuição clínica e etiológica.
        Hospital. 1943; 24: 49-63
        • Williamson P.M.
        Neuromyelitis optica following infectious mononucleosis.
        Proc Aust Assoc Neurol. 1975; 12: 153-155
        • Papais-Alvarenga
        • Miranda-Santos C.M.
        • Puccioni-Sohler M.
        • de Almeida A.M.
        • Oliveira S.
        • de Oliveira CA Basilio
        • et al.
        Optic neuromyelitis syndrome in Brazilian patients.
        J Neurol Neurosurg Psychiatry. 2002; 73: 429-435
        • Miranda de Sousa A.
        • Puccioni-Sohler M.
        • Dias Borges A.
        • Fernandez Adorno L.
        • Papais Alvarenga M.
        • Papis Alvarenga R.M.
        Post-dengue neuromyelitis optica: case report of a Japanese-descendant Brazilian child.
        J Infect Chemother. 2006; 12: 396e8
        • Wilcox R.A.
        • Burrow J.
        • Slee M.
        • Craig J.
        • Thyagarajan D.
        Neuromyelitis optica (Devic's disease) in a patient with syphilis.
        Mult Scler. 2008; 14: 268e71
        • Blanche P.
        • Diaz E.
        • Gombert B.
        • Sicard D.
        Devic's neuromyelitis optica and HIV-1 infection.
        J Neurol Neurosurg Psychiatry. 2000; 68: 795-796
        • Tsubaki T.
        • Honma Y.
        • Hoshi M.
        Neurological syndrome associated with clioquinol.
        Lancet. 1971; 1: 696-697
        • Banwell B.
        • tenemboum S.
        • Lennon V.A.
        • Ursell E.
        • Kennedy J.
        • bar-Or A.
        • et al.
        Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorder.
        Neurology. 2008; 70: 344-352
        • Lotze T.E.
        • Northrop J.L.
        • Hutton G.J.
        • Ross B.
        • schiffman J.S.
        • Hunter J.V.
        Spectrum of pediatric neuromyelitis optica.
        Pediatrics. 2008; 122: e1039-e1047
        • Oktarina D.A.
        • van der Wier G.
        • Diercks G.F.
        • Jonkman M.F.
        • Pas H.H.
        IgG-induced clustering of desmogleins 1 and 3 in skin of patients with pemphigus fits with the desmoglein nonassembly depletion hypothesis.
        Br J Dermatol. Sep 2011; 165: 552-562
        • Amagai M.
        • Stanley J.R.
        Desmoglein as a target in skin disease and beyond.
        J Invest Dermatol. Mar 2012; 132: 776-784
        • Grando S.A.
        • Holubar K.
        • Schwartz R.A.
        Jean-Claude Bystryn 1938–2010. An obituary.
        Exp Dermatol. Mar 2011; 20: 293-296
        • Lennon V.A.
        • Wingerchuk D.M.
        • Kryzer T.J.
        • Pittock S.J.
        • Lucchinetti C.F.
        • Fujihara K.
        • et al.
        A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis.
        Lancet. 2004; 364: 2106e12
        • Tait M.J.
        • saadoun S.
        • Bell B.A.
        • Papadopoulos M.C.
        Water movements in the brain: role of aquaporins.
        Trends Neurosci. 2008; 31: 37-43
        • Hara-Chikuma M.
        • Verkman A.S.
        Aquaporin-3 functions as a glycerol transporter in mammalian skin.
        Biol Cell. 2005; 97: 479-486
        • Boury-Jamot M.
        • Daraspe J.
        • Bonte F.
        • Perrier E.
        • Schnebert S.
        • Dumas M.
        • et al.
        Skin aquaporins: function in hydration, wound healing and skin epidermis homeostasis.
        Handb Exp Pharmacol. 2009; 190: 205-217
        • Hara-Chikuma M.
        • Verkman A.S.
        Roles of aquaporin-3 in the epidermis.
        J Invest Dermatol. Sep 2008; 128: 2145-2151
        • Suárez-Fariñas M.
        • Fuentes-Duculan J.
        • Lowes M.A.
        • Krueger J.G.
        Resolved psoriasis lesions retain expression of a subset of disease-related genes.
        J Invest Dermatol. Feb 2011; 131: 391-400
        • Hara-Chikuma M.
        • Sugiyama Y.
        • Kabashima K.
        • Sohara E.
        • Uchida S.
        • Sasaki S.
        • et al.
        Involvement of aquaporin-7 in the cutaneous primary immune response through modulation of antigen uptake and migration in dendritic cells.
        FASEB J. Jan 2012; 26: 211-218
        • Hara-Chikuma M.
        • Verkman A.S.
        Aquaporin-3 facilitates epidermal cell migration and proliferation during wound healing.
        J Mol Med. 2008; 86: 221-231
        • Ma T.
        • Hara M.
        • Sougrat R.
        • Verbavatz J.M.
        • Verkman A.S.
        Impaired stratum corneum hydration in mice lacking epidermal water channel aquaporin-3.
        J Biol Chem. 2002; 277: 17147-17153
        • Marchini G.
        • Ståbi B.
        • Kankes K.
        • Lonne-Rahm S.
        • Østergaard M.
        • Nielsen S.
        AQP1 and AQP3, psoriasin, and nitric oxide synthases 1–3 are inflammatory mediators in erythema toxicum neonatorum.
        Pediatr Dermatol. 2003; 20: 377-384
        • Straseski J.A.
        • Gibson A.L.
        • Thomas-Virnig C.L.
        • Allen-Hoffmann B.L.
        Oxygen deprivation inhibits basal keratinocyte proliferation in a model of human skin and induces regio-specific changes in the distribution of epidermal adherens junction proteins, aquaporin-3, and glycogen.
        Wound Repair Regen. 2009; 17: 606-616
        • Matsuya N.
        • Komori M.
        • Nomura K.
        • Nakane S.
        • Fukudome T.
        • Goto H.
        • et al.
        Increased T-cell immunity against aquaporin-4 and proteolipid protein in neuromyelitis optica.
        Int Immunol. 2011; 23: 565-573
        • Kim W.
        • Kim S.H.
        • Kim H.J.
        New insights into neuromyelitis optica.
        J Clin Neurol. 2011; 7: 115-127
        • Pohl M.
        • Fischer M.T.
        • Mader S.
        • Schanda K.
        • Kitic M.
        • Sharma R.
        • et al.
        Pathogenic T cell responses against aquaporin 4.
        Acta Neuropathol. Jul 2011; 122: 21-34
        • Nishifuji K.
        • Amagai M.
        • Kuwana M.
        • Iwasaki T.
        • Nishikawa T.
        Detection of antigen-specific B cells in patients with pemphigus vulgaris by enzyme-linked immunospot assay: requirement of T cell collaboration for autoantibody production.
        J Invest Dermatol. Jan 2000; 114: 88-94
        • Wada N.
        • Nishifuji K.
        • Yamada T.
        • Kudoh J.
        • Shimizu N.
        • Matsumoto M.
        • et al.
        Aire-dependent thymic expression of desmoglein 3, the autoantigen in pemphigus vulgaris, and its role in T-cell tolerance.
        J Invest Dermatol. Feb 2011; 131: 410-417
        • Culton D.A.
        • Qian Y.
        • Li N.
        • Rubenstein D.
        • Aoki V.
        • Filhio G.H.
        • et al.
        Advances in pemphigus and its endemic pemphigus foliaceus (Fogo Selvagem) phenotype: a paradigm of human autoimmunity.
        J Autoimmun. Dec 2008; 31: 311-324
        • Beissert S.
        • Werfel T.
        • Frieling U.
        • Böhm M.
        • Sticherling M.
        • Stadler R.
        • et al.
        A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus.
        Arch Dermatol. Nov 2006; 142: 1447-1454
        • Papeix C.
        • Vidal J.S.
        • de Seze J.
        • Pierrot-Deseilligny C.
        • Tourbah A.
        • Stankoff B.
        • et al.
        Immunosuppressive therapy is more effective than interferon in neuromyelitis optica.
        Mult Scler. 2007; 13: 256e9
        • Wingerchuk D.M.
        Diagnosis and treatment of neuromyelitis optica.
        Neurologist. Jan 2007; 13: 2-11
        • Herr A.L.
        • Hatami A.
        • Kokta V.
        • Dalle J.H.
        • Champagne M.A.
        • Duval M.
        Successful anti-CD20 antibody treatment of pemphigus foliaceus after unrelated cord blood transplantation.
        Bone Marrow Transplant. Feb 2005; 35: 427-428
        • Jacob A.
        • Weinshenker B.G.
        • Violich I.
        • McLinskey N.
        • Krupp L.
        • Fox R.J.
        • et al.
        Treatment of neuromyelitis optic with rituximab: retrospective analysis of 25 patients.
        Arch Neurol. 2008; 65: 1443e8