Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma

  • Asya I. Wallach
  • Cynthia M. Magro
  • Andrew G. FranksJr
  • Lee Shapiro
  • Ilya Kister
Neurology of Systemic Diseases (J Biller, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Neurology of Systemic Disease


Purpose of Review

To describe diverse neurologic and neuroradiologic presentations of two rare, immunologically mediated skin conditions: Sweet disease and localized scleroderma (morphea).

Recent Findings

Core syndromes of neuro-Sweet disease (NSD) are steroid responsiveness, recurrent meningitis, and encephalitis. Focal neurologic, neuro-vascular, and neuro-ophthalmologic syndromes have been reported recently in NSD. A variety of steroid-sparing treatments and biologics have been used for relapsing NSD. Localized craniofacial scleroderma is associated with seizures, headaches, and, less commonly, focal deficits and cognitive decline. Immunosuppressive therapy may be required in patients with disease progression; some refractory cases have responded to IL-6 inhibition.


Our review provides an up-to-date reference for neurologists faced with a patient with a history or skin findings consistent with Sweet disease or localized scleroderma. We hope that it will stimulate collaborative studies aimed at unraveling the pathogenesis of these disorders, better characterization of their neurologic manifestations, and discovery of optimal therapeutic solutions.


Sweet syndrome Neuro-Sweet disease Localized scleroderma Progressive hemifacial atrophy Anti-Il 6 therapy Neurologic complications 


Compliance with Ethical Standards

Conflict of Interest

Asya Wallach reports educational grants from the National MS Society and Biogen. Lee Shapiro reports he served on advisory board for Genentech. Ilya Kister reports he served on advisory boards for Biogen and Genentech and received research support for investigator-initiated grants from Sanofi Genzyme, Biogen, EMD Serono, National MS Society, and Guthy Jackson Charitable Foundation. Cynthia Magro and Andrew Franks each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Hurko O, Provost TT. Neurology and the skin. J Neurol Neurosurg Psychiatry. 1999;66(4):417–30.CrossRefGoogle Scholar
  2. 2.
    Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M. Neutrophilic dermatoses: pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behcet disease. J Am Acad Dermatol. 2018;79(6):987–1006. Scholar
  3. 3.
    Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. J Am Acad Dermatol. 2000;43(5 Pt 1):870–4. Scholar
  4. 4.
    •• Drago F, Ciccarese G, Agnoletti AF, Sarocchi F, Parodi A. Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome. Acta Neurol Belg. 2017;117(1):33–42. Excellent systematic review of Neuro Sweet syndrome, including imaging, pathology and treatment approach. CrossRefPubMedGoogle Scholar
  5. 5.
    Kandula S, Burke WS, Goldfarb JN. Clindamycin-induced Sweet syndrome. J Am Acad Dermatol. 2010;62(5):898–900. Scholar
  6. 6.
    Thompson DF, Montarella KE. Drug-induced Sweet’s syndrome. Ann Pharmacother. 2007;41(5):802–11. Scholar
  7. 7.
    Clark BM, Homeyer DC, Glass KR, D'Avignon LC. Clindamycin-induced Sweet’s syndrome. Pharmacotherapy. 2007;27(9):1343–6. Scholar
  8. 8.
    Cohen PR. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol. 2009;10(5):301–12. Scholar
  9. 9.
    Hisanaga K, Hosokawa M, Sato N, Mochizuki H, Itoyama Y, Iwasaki Y. “Neuro-Sweet disease”: benign recurrent encephalitis with neutrophilic dermatosis. Arch Neurol. 1999;56(8):1010–3.CrossRefGoogle Scholar
  10. 10.
    Hisanaga K, Iwasaki Y, Itoyama Y. Neuro-Sweet Disease Study G. Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology. 2005;64(10):1756–61. Scholar
  11. 11.
    Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS, et al. Diagnosis and management of neuro-Behcet’s disease: international consensus recommendations. J Neurol. 2014;261(9):1662–76. Scholar
  12. 12.
    Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behcet’s disease: evaluation of 200 patients. The Neuro-Behcet Study Group. Brain. 1999;122(Pt 11):2171–82.CrossRefGoogle Scholar
  13. 13.
    Kato T, Kunikata N, Taira H, Kobayashi N, Tanji K, Endo M. Acute febrile neutrophilic dermatosis (Sweet’s syndrome) with nodular episcleritis and polyneuropathy. Int J Dermatol. 2002;41(2):107–9.CrossRefGoogle Scholar
  14. 14.
    Cala CM, Kole L, Sami N. Bilateral sensorineural hearing loss and polyneuropathy in a patient with Sweet’s syndrome. Case Rep Otolaryngol. 2015;2015:751538–3. Scholar
  15. 15.
    Taravati P. Neuro-sweet disease causing orbital inflammation. Neuroophthalmology. 2015;39(1):42–5. Scholar
  16. 16.
    Lobo AM, Stacy R, Cestari D, Stone JH, Jakobiec FA, Sobrin L. Optic nerve involvement with panuveitis in Sweet syndrome. Ocul Immunol Inflamm. 2011;19(3):167–70. Scholar
  17. 17.
    Sobol UA, Sherman KL, Smith J, Nagda SN, Micetich K, Nickoloff BJ, et al. Sweet’s syndrome with neurologic manifestations in a patient with esophageal adenocarcinoma: case report and review of the literature. Int J Dermatol. 2009;48(10):1062–5.CrossRefGoogle Scholar
  18. 18.
    Ohori N, Kinoshita T, Toda K, Ohta S, Yoshimura T. A case of Sweet’s syndrome (acute febrile neutrophilic dermatosis) showing transient jargon aphasia. Rinsho Shinkeigaku. 1999;39(11):1156–9.PubMedGoogle Scholar
  19. 19.
    Macorig G, Pessa ME, Barbarino G, Scalise S, D'Agostini S, Valente M, et al. Neuro-Sweet disease: a diagnostic challenge. J Neurol Sci. 2016;371:42–4. Scholar
  20. 20.
    Makimoto G, Manabe Y, Yamakawa C, Fujii D, Ikeda-Sakai Y, Narai H, et al. Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation. Neurol Int. 2012;4(1):e5. Scholar
  21. 21.
    Niwa F, Tokuda T, Kimura M, Azuma Y, Mizuno T, Nakagawa M. Self-remitting and reversible parkinsonism associated with neuro-Sweet disease. Intern Med. 2010;49(12):1201–4.CrossRefGoogle Scholar
  22. 22.
    Fukushima K, Hineno A, Kodaira M, Machida K, Ishii W, Kaneko T, et al. Reversible extensive leukoencephalopathy in Sweet disease: a case report. J Neurol Sci. 2008;275(1–2):178–80. Scholar
  23. 23.
    Kimura A, Sakurai T, Koumura A, Suzuki Y, Tanaka Y, Hozumi I, et al. Longitudinal analysis of cytokines and chemokines in the cerebrospinal fluid of a patient with neuro-Sweet disease presenting with recurrent encephalomeningitis. Intern Med. 2008;47(3):135–41.CrossRefGoogle Scholar
  24. 24.
    Marien P, Tops W, Crols R, Jonkers R, De Deyn PP, Verhoeven J. Grammar disruption in a patient with neuro-Sweet syndrome. Neurocase. 2012;18(3):235–47. Scholar
  25. 25.
    Das AS, Conway SE, Unizony SH, Bouffard MA, Rost NS, Venna N. Pearls & oysters: neuro-Sweet disease presenting as ischemic stroke and aseptic meningitis. Neurology. 2018;91(23):e2197–e9. Scholar
  26. 26.
    Sakamoto M, Kurimoto T, Mori S, Ueda K, Keshi Y, Yamada Y, et al. Vasculitis with superior ophthalmic vein thrombosis compatible with neuro-neutrophilic disease. Am J Ophthalmol Case Rep. 2018;12:39–44. Scholar
  27. 27.
    Sato K, Tsunoda K, Yamashita T, Takemoto M, Hishikawa N, Ohta Y, et al. A case of very long longitudinally extensive transverse myelitis (LETM) with necrotizing Vasculitis. J Neurol Sci. 2017;373:152–4. Scholar
  28. 28.
    Sudhakar P, Tobin S, Connor WO, Kedar S. Neuro-ophthalmic presentation of neuro-Sweet disease. Neuroophthalmology. 2017;41(4):202–6. Scholar
  29. 29.
    Maxwell G, Archibald N, Turnbull D. Neuro-Sweet’s disease. Pract Neurol. 2012;12(2):126–30. Scholar
  30. 30.
    Magro CM, De Moraes E, Burns F. Sweet’s syndrome in the setting of CD34-positive acute myelogenous leukemia treated with granulocyte colony stimulating factor: evidence for a clonal neutrophilic dermatosis. J Cutan Pathol. 2001;28(2):90–6.CrossRefGoogle Scholar
  31. 31.
    • Charlson R, Kister I, Kaminetzky D, Shvartsbeyn M, Meehan SA, Mikolaenko I. CNS neutrophilic vasculitis in neuro-Sweet disease. Neurology. 2015;85(9):829–30. Neuropathologic findings in acute parenchymal brain lesion of Newuo-sweet disease. CrossRefPubMedGoogle Scholar
  32. 32.
    Kokubo Y, Kuzuhara S, Isoda K, Sato K, Kawada N, Narita Y. Neuro-Sweet disease: report of the first autopsy case. J Neurol Neurosurg Psychiatry. 2007;78(9):997–1000. Scholar
  33. 33.
    Akiba C, Esaki T, Ando M, Furuya T, Noda K, Nakao Y, et al. Possible neuro-Sweet disease mimicking brain tumor in the medulla oblongata--case report. Neurol Med Chir (Tokyo). 2011;51(2):140–3.CrossRefGoogle Scholar
  34. 34.
    Nakanishi E, Sawamura M, Maruhama S, Yamada H, Kim G, Harada K. Neuro-neutrophilic disease suspected by human leukocyte antigen (HLA) typing and brain biopsy: a case report. Rinsho Shinkeigaku. 2015;55(1):13–7. Scholar
  35. 35.
    Magro CM, Momtahen S, Nguyen GH, Wang X. Histiocytoid Sweet’s syndrome: a localized cutaneous proliferation of macrophages frequently associated with chronic myeloproliferative disease. Eur J Dermatol. 2015;25(4):335–41. Scholar
  36. 36.
    Delluc A, Limal N, Puechal X, Frances C, Piette JC, Cacoub P. Efficacy of anakinra, an IL1 receptor antagonist, in refractory Sweet syndrome. Ann Rheum Dis. 2008;67(2):278–9. Scholar
  37. 37.
    Prat L, Bouaziz JD, Wallach D, Vignon-Pennamen MD, Bagot M. Neutrophilic dermatoses as systemic diseases. Clin Dermatol. 2014;32(3):376–88. Scholar
  38. 38.
    Seminario-Vidal L, Guerrero C, Sami N. Refractory Sweet’s syndrome successfully treated with rituximab. JAAD Case Rep. 2015;1(3):123–5. Scholar
  39. 39.
    Keidel S, McColl A, Edmonds S. Sweet’s syndrome after adalimumab therapy for refractory relapsing polychondritis. BMJ Case Rep. 2011;2011.
  40. 40.
    • Maalouf D, Battistella M, Bouaziz JD. Neutrophilic dermatosis: disease mechanism and treatment. Curr Opin Hematol. 2015;22(1):23–9. Review of therapeutic options, including anti-IL-1 therapy for neutrophilic dermatoses. CrossRefPubMedGoogle Scholar
  41. 41.
    • Pinho J, Rocha J, Sousa F, Macedo C, Soares-Fernandes J, Cerqueira J, et al. Localized scleroderma en coup de sabre in the neurology clinic. Mult Scler Relat Disord. 2016;8:96–8. Five detailed neurological cases with MRIs findings. CrossRefPubMedGoogle Scholar
  42. 42.
    Kraus V, Lawson EF, von Scheven E, Tihan T, Garza J, Nathan RG, et al. Atypical cases of scleroderma en coup de sabre. J Child Neurol. 2014;29(5):698–703. Scholar
  43. 43.
    Rongioletti F, Ferreli C, Atzori L, Bottoni U, Soda G. Scleroderma with an update about clinico-pathological correlation. G Ital Dermatol Venereol. 2018;153(2):208–15. Scholar
  44. 44.
    Gordon JK, Martyanov V, Magro C, Wildman HF, Wood TA, Huang WT, et al. Nilotinib (Tasigna) in the treatment of early diffuse systemic sclerosis: an open-label, pilot clinical trial. Arthritis Res Ther. 2015;17:213. Scholar
  45. 45.
    Gordon J, Udeh U, Doobay K, Magro C, Wildman H, Davids M, et al. Imatinib mesylate (Gleevec) in the treatment of diffuse cutaneous systemic sclerosis: results of a 24-month open label, extension phase, single-centre trial. Clin Exp Rheumatol. 2014;32(6 Suppl 86):S-189–93.Google Scholar
  46. 46.
    Karaca NE, Aksu G, Karaca E, Tuzun F, Gunes AT, Ozkinay F, et al. Progressive morphea of early childhood tracing Blaschko’s lines on the face: involvement of X chromosome monosomy in pathogenesis and clinical prognosis. Int J Dermatol. 2011;50(11):1406–10. Scholar
  47. 47.
    Leitenberger JJ, Cayce RL, Haley RW, Adams-Huet B, Bergstresser PR, Jacobe HT. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol. 2009;145(5):545–50. Scholar
  48. 48.
    Jacobe H, Ahn C, Arnett FC, Reveille JD. Major histocompatibility complex class I and class II alleles may confer susceptibility to or protection against morphea: findings from the Morphea in adults and children cohort. Arthritis Rheumatol. 2014;66(11):3170–7. Scholar
  49. 49.
    Pham AK, Srivastava B, Deng A. Pregnancy-associated morphea: a case report and literature review. Dermatol Online J. 2017;23(1).Google Scholar
  50. 50.
    Christianson HB, Dorsey CS, Kierland RR, O'Leary PA. Localized scleroderma; a clinical study of two hundred thirty-five cases. AMA Arch Derm. 1956;74(6):629–39.CrossRefGoogle Scholar
  51. 51.
    Khamaganova I. Localized scleroderma: predisposing and triggering factors. Open Dermatol J 2017;11:1–11.
  52. 52.
    Fruchter R, Kurtzman DJB, Mazori DR, Wright NA, Patel M, Vleugels RA, et al. Characteristics and treatment of postirradiation morphea: a retrospective multicenter analysis. J Am Acad Dermatol. 2017;76(1):19–21. Scholar
  53. 53.
    De Somer L, Morren MA, Muller PC, Despontin K, Jansen K, Lagae L, et al. Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort. Eur J Pediatr. 2015;174(9):1247–54. Scholar
  54. 54.
    Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45(5):614–20. Scholar
  55. 55.
    Magro CM, Ross P, Marsh CB, Allen JN, Liff D, Knight DA, et al. The role of anti-endothelial cell antibody-mediated microvascular injury in the evolution of pulmonary fibrosis in the setting of collagen vascular disease. Am J Clin Pathol. 2007;127(2):237–47. Scholar
  56. 56.
    Wusirika R, Ferri C, Marin M, Knight DA, Waldman WJ, Ross P Jr, et al. The assessment of anti-endothelial cell antibodies in scleroderma-associated pulmonary fibrosis. A study of indirect immunofluorescent and western blot analysis in 49 patients with scleroderma. Am J Clin Pathol. 2003;120(4):596–606. Scholar
  57. 57.
    Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70(11):1068–76. Scholar
  58. 58.
    • Wong M, Phillips CD, Hagiwara M, Shatzkes DR. Parry Romberg syndrome: 7 cases and literature review. AJNR Am J Neuroradiol. 2015;36(7):1355–61. Seven neurological patient cases with relevant neuroimaging. CrossRefPubMedGoogle Scholar
  59. 59.
    Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64(2):217–28; quiz 29-30. Scholar
  60. 60.
    Kister I, Inglese M, Laxer RM, Herbert J. Neurologic manifestations of localized scleroderma: a case report and literature review. Neurology. 2008;71(19):1538–45. Scholar
  61. 61.
    Sommer A, Gambichler T, Bacharach-Buhles M, von Rothenburg T, Altmeyer P, Kreuter A. Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol. 2006;54(2):227–33. Scholar
  62. 62.
    Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007;56(2):257–63. Scholar
  63. 63.
    Chiu YE, Vora S, Kwon EK, Maheshwari M. A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol. 2012;29(6):738–48. Scholar
  64. 64.
    Christen-Zaech S, Hakim MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008;59(3):385–96. Scholar
  65. 65.
    • Mansour M, Liy Wong C, Zulian F, Li S, Morishita K, Yeh EA, et al. Natural history and extracutaneous involvement of congenital morphea: Multicenter retrospective cohort study and literature review. Pediatr Dermatol. 2018;35(6):761–8. Different types of morphea found in neonates (congenital morphea) are reviewed including clinical characteristics, extracutaneous manifestations and laboratory markers. CrossRefPubMedGoogle Scholar
  66. 66.
    Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum. 2013;43(3):335–47. Scholar
  67. 67.
    Carreno M, Donaire A, Barcelo MI, Rumia J, Falip M, Agudo R, et al. Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis. Neurology. 2007;68(16):1308–10. Scholar
  68. 68.
    Doolittle DA, Lehman VT, Schwartz KM, Wong-Kisiel LC, Lehman JS, Tollefson MM. CNS imaging findings associated with Parry-Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities. Neuroradiology. 2015;57(1):21–34. Scholar
  69. 69.
    • Maloney E, Menashe SJ, Iyer RS, Ringold S, Chakraborty AK, Ishak GE. The central nervous system manifestations of localized craniofacial scleroderma: a study of 10 cases and literature review. Pediatr Radiol. 2018;48(11):1642–54. CNS manifestations of craniofacial scleroderma and impressive imaging findings, including single case of PET/CT in a morphea patient. CrossRefPubMedGoogle Scholar
  70. 70.
    Fain ET, Mannion M, Pope E, Young DW, Laxer RM, Cron RQ. Brain cavernomas associated with en coup de sabre linear scleroderma: two case reports. Pediatr Rheumatol Online J. 2011;9:18. Scholar
  71. 71.
    Stone J, Franks AJ, Guthrie JA, Johnson MH. Scleroderma “en coup de sabre”: pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry. 2001;70(3):382–5.CrossRefGoogle Scholar
  72. 72.
    English SW, Ho ML, Tollefson MM, Wong-Kisiel LC. Focal epilepsy in a teenager with facial atrophy and hair loss. Semin Pediatr Neurol. 2018;26:68–73. Scholar
  73. 73.
    Rimoin L, Arbiser J. Improvement of “en coup de sabre” morphea and associated headaches with botulinum toxin injections. Dermatol Surg. 2016;42(10):1216–9. Scholar
  74. 74.
    Infante-Valenzuela A, Camara-Lemarroy CR, Delgado-Garcia G, Gil-Valdez AH, Marfil-Rivera A. Steroid-responsive headache in scleroderma en coup de sabre. Acta Neurol Belg. 2017;117(1):405–7. Scholar
  75. 75.
    Polcari I, Moon A, Mathes EF, Gilmore ES, Paller AS. Headaches as a presenting symptom of linear morphea en coup de sabre. Pediatrics. 2014;134(6):e1715–9. Scholar
  76. 76.
    Camacho-Velasquez JL. Nummular headache associated with linear scleroderma. Headache. 2016;56(9):1492–3. Scholar
  77. 77.
    Lenassi E, Vassallo G, Kehdi E, Chieng AS, Ashworth JL. Craniofacial linear scleroderma associated with retinal telangiectasia and exudative retinal detachment. J AAPOS. 2017;21(3):251–4. Scholar
  78. 78.
    Walker RH, Fink JK. Morphea and Parry-Romberg syndrome associated with a mixed movement disorder. Parkinsonism Relat Disord. 2013;19(12):1169–70. Scholar
  79. 79.
    Sathornsumetee S, Schanberg L, Rabinovich E, Lewis D Jr, Weisleder P. Parry-Romberg syndrome with fatal brain stem involvement. J Pediatr. 2005;146(3):429–31. Scholar
  80. 80.
    Klimiec E, Klimkowicz-Mrowiec A. Mild cognitive impairment as a single sign of brain hemiatrophy in patient with localized scleroderma and Parry-Romberg syndrome. Neurol Neurochir Pol. 2016;50(3):215–8. Scholar
  81. 81.
    Miyamoto M, Kinoshita M, Tanaka K, Tanaka M. Recurrent myelitis in localized scleroderma. Clin Neurol Neurosurg. 2014;127:140–2. Scholar
  82. 82.
    Kanzato N, Matsuzaki T, Komine Y, Saito M, Saito A, Yoshio T, et al. Localized scleroderma associated with progressing ischemic stroke. J Neurol Sci. 1999;163(1):86–9.CrossRefGoogle Scholar
  83. 83.
    Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. 2006;117(1):e132–6. Scholar
  84. 84.
    Chung MH, Sum J, Morrell MJ, Horoupian DS. Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings. Ann Neurol. 1995;37(5):679–81. Scholar
  85. 85.
    Takahashi T, Asano Y, Oka T, Miyagaki T, Tamaki Z, Nonaka S, et al. Scleroderma en coup de sabre with recurrent episodes of brain hemorrhage. J Dermatol. 2016;43(2):203–6. Scholar
  86. 86.
    Franklin ML, Day S. Bilateral choroidal excavation in juvenile localized scleroderma. Retin Cases Brief Rep. 2018;12(1):21–3. Scholar
  87. 87.
    Zannin ME, Martini G, Athreya BH, Russo R, Higgins G, Vittadello F, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol. 2007;91(10):1311–4. Scholar
  88. 88.
    Laxer RM, Miller S, Pope E. Adie pupil as the initial presentation of localized en coup de sabre scleroderma. J Rheumatol. 2017;44(7):1096–7. Scholar
  89. 89.
    Hock LE, Kontzialis M, Szewka AJ. Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. Neurology. 2016;87(16):1741–2. Scholar
  90. 90.
    Fledelius HC, Danielsen PL, Ullman S. Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. Eye (Lond). 2018;32(11):1688–96. Scholar
  91. 91.
    Rosario C, Garelick D, Greenberg G, Chapman J, Shoenfeld Y, Langevitz P. Plaque morphea with neurological involvement-an extraordinary uncommon presentation. Clin Rheumatol. 2015;34(3):597–601. Scholar
  92. 92.
    Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, et al. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res (Hoboken). 2012;64(8):1175–85. Scholar
  93. 93.
    Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, et al. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol. 2017;31(9):1401–24. Scholar
  94. 94.
    Osminina M, Geppe N, Afonina E. Scleroderma “en coup de sabre” with epilepsy and uveitis successfully treated with tocilizumab. Reumatol Clin. 2018.
  95. 95.
    Magro CM, Halteh P, Olson LC, Shapiro L, Kister I. Linear scleroderma with brain involvement—Clinicopathologic analysis and response to tocilizumab. Orphanet J Rare Dis. (2019);(in press).Google Scholar
  96. 96.
    • Lythgoe H, Baildam E, Beresford MW, Cleary G, McCann LJ, Pain CE. Tocilizumab as a potential therapeutic option for children with severe, refractory juvenile localized scleroderma. Rheumatology (Oxford). 2018;57(2):398–401. Case series of 5 patients with localized scleroderma, demonstrates safety and presumed efficacy of tocilizumab for severe refractory disease. CrossRefGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Asya I. Wallach
    • 1
  • Cynthia M. Magro
    • 2
  • Andrew G. FranksJr
    • 3
    • 4
  • Lee Shapiro
    • 5
  • Ilya Kister
    • 1
  1. 1.NYU Multiple Sclerosis Comprehensive Care Center, Department of NeurologyNew York University School of MedicineNew YorkUSA
  2. 2.Department of Pathology and Laboratory MedicineWeill Cornell MedicineNew YorkUSA
  3. 3.Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of DermatologyNew York University School of MedicineNew YorkUSA
  4. 4.Division of Rheumatology, Department of MedicineNew York University School of MedicineNew YorkUSA
  5. 5.Community Care RheumatologySaratoga SpringsUSA

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