Skip to main content

Advertisement

SpringerLink
Log in

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

  • Neurology of Systemic Diseases (J Biller, Section Editor)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

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.

Summary

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

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

  1. Hurko O, Provost TT. Neurology and the skin. J Neurol Neurosurg Psychiatry. 1999;66(4):417–30.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1016/j.jaad.2017.11.064.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1067/mjd.2000.109286.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s13760-016-0695-1. Excellent systematic review of Neuro Sweet syndrome, including imaging, pathology and treatment approach.

    Article  PubMed  Google Scholar 

  5. Kandula S, Burke WS, Goldfarb JN. Clindamycin-induced Sweet syndrome. J Am Acad Dermatol. 2010;62(5):898–900. https://doi.org/10.1016/j.jaad.2009.03.050.

    Article  PubMed  Google Scholar 

  6. Thompson DF, Montarella KE. Drug-induced Sweet’s syndrome. Ann Pharmacother. 2007;41(5):802–11. https://doi.org/10.1345/aph.1H563.

    Article  CAS  PubMed  Google Scholar 

  7. Clark BM, Homeyer DC, Glass KR, D'Avignon LC. Clindamycin-induced Sweet’s syndrome. Pharmacotherapy. 2007;27(9):1343–6. https://doi.org/10.1592/phco.27.9.1343.

    Article  CAS  PubMed  Google Scholar 

  8. Cohen PR. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol. 2009;10(5):301–12. https://doi.org/10.2165/11310730-000000000-00000.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1212/01.WNL.0000161848.34159.B5.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s00415-013-7209-3.

    Article  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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. https://doi.org/10.1155/2015/751538.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Taravati P. Neuro-sweet disease causing orbital inflammation. Neuroophthalmology. 2015;39(1):42–5. https://doi.org/10.3109/01658107.2014.961090.

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.3109/09273948.2011.560411.

    Article  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.jns.2016.10.012.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.4081/ni.2012.e5.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  Google Scholar 

  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. https://doi.org/10.1016/j.jns.2008.08.006.

    Article  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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. https://doi.org/10.1080/13554794.2011.588178.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1212/WNL.0000000000006628.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.ajoc.2018.08.005.

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1016/j.jns.2016.12.040.

    Article  PubMed  Google Scholar 

  28. Sudhakar P, Tobin S, Connor WO, Kedar S. Neuro-ophthalmic presentation of neuro-Sweet disease. Neuroophthalmology. 2017;41(4):202–6. https://doi.org/10.1080/01658107.2017.1291687.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Maxwell G, Archibald N, Turnbull D. Neuro-Sweet’s disease. Pract Neurol. 2012;12(2):126–30. https://doi.org/10.1136/practneurol-2011-000067.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1212/WNL.0000000000001892. Neuropathologic findings in acute parenchymal brain lesion of Newuo-sweet disease.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1136/jnnp.2006.105650.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  Google Scholar 

  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. https://doi.org/10.5692/clinicalneurol.55.13.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1684/ejd.2015.2586.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1136/ard.2006.068254.

    Article  CAS  PubMed  Google Scholar 

  37. Prat L, Bouaziz JD, Wallach D, Vignon-Pennamen MD, Bagot M. Neutrophilic dermatoses as systemic diseases. Clin Dermatol. 2014;32(3):376–88. https://doi.org/10.1016/j.clindermatol.2013.11.004.

    Article  PubMed  Google Scholar 

  38. Seminario-Vidal L, Guerrero C, Sami N. Refractory Sweet’s syndrome successfully treated with rituximab. JAAD Case Rep. 2015;1(3):123–5. https://doi.org/10.1016/j.jdcr.2015.03.002.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Keidel S, McColl A, Edmonds S. Sweet’s syndrome after adalimumab therapy for refractory relapsing polychondritis. BMJ Case Rep. 2011;2011. https://doi.org/10.1136/bcr.10.2011.4935.

  40. • Maalouf D, Battistella M, Bouaziz JD. Neutrophilic dermatosis: disease mechanism and treatment. Curr Opin Hematol. 2015;22(1):23–9. https://doi.org/10.1097/MOH.0000000000000100. Review of therapeutic options, including anti-IL-1 therapy for neutrophilic dermatoses.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.msard.2016.05.013. Five detailed neurological cases with MRIs findings.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1177/0883073813520494.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.23736/S0392-0488.18.05922-9.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1186/s13075-015-0721-3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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. 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. https://doi.org/10.1111/j.1365-4632.2011.04900.x.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1001/archdermatol.2009.79.

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1002/art.38814.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Pham AK, Srivastava B, Deng A. Pregnancy-associated morphea: a case report and literature review. Dermatol Online J. 2017;23(1).

  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.

    Article  CAS  Google Scholar 

  51. Khamaganova I. Localized scleroderma: predisposing and triggering factors. Open Dermatol J 2017;11:1–11. https://doi.org/10.2174/1874372201711010001.

  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. https://doi.org/10.1016/j.jaad.2016.08.059.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s00431-015-2532-6.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1093/rheumatology/kei251.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1309/CNQDMHLH2WGKL32T.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1309/8HVC-MJMY-NPUQ-PBD2.

    Article  CAS  PubMed  Google Scholar 

  57. Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70(11):1068–76. https://doi.org/10.1016/S0025-6196(11)64442-X.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.3174/ajnr.A4297. Seven neurological patient cases with relevant neuroimaging.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.jaad.2010.05.045.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1212/01.wnl.0000334474.88923.e3.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.jaad.2005.10.020.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.jaad.2006.10.959.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1111/pde.12001.

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1016/j.jaad.2008.05.005.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1111/pde.13605. Different types of morphea found in neonates (congenital morphea) are reviewed including clinical characteristics, extracutaneous manifestations and laboratory markers.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.semarthrit.2013.05.002.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1212/01.wnl.0000259523.09001.7a.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s00234-014-1448-6.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s00247-018-4177-x. CNS manifestations of craniofacial scleroderma and impressive imaging findings, including single case of PET/CT in a morphea patient.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1186/1546-0096-9-18.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1016/j.spen.2017.03.009.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1097/DSS.0000000000000812.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1007/s13760-016-0673-7.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1542/peds.2014-0019.

    Article  PubMed  Google Scholar 

  76. Camacho-Velasquez JL. Nummular headache associated with linear scleroderma. Headache. 2016;56(9):1492–3. https://doi.org/10.1111/head.12894.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.jaapos.2016.12.004.

    Article  PubMed  Google Scholar 

  78. Walker RH, Fink JK. Morphea and Parry-Romberg syndrome associated with a mixed movement disorder. Parkinsonism Relat Disord. 2013;19(12):1169–70. https://doi.org/10.1016/j.parkreldis.2013.07.025.

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.1016/j.jpeds.2004.10.026.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1016/j.pjnns.2016.02.002.

    Article  PubMed  Google Scholar 

  81. Miyamoto M, Kinoshita M, Tanaka K, Tanaka M. Recurrent myelitis in localized scleroderma. Clin Neurol Neurosurg. 2014;127:140–2. https://doi.org/10.1016/j.clineuro.2014.10.005.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1542/peds.2005-0470.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1002/ana.410370519.

    Article  CAS  PubMed  Google Scholar 

  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. https://doi.org/10.1111/1346-8138.13023.

    Article  PubMed  Google Scholar 

  86. Franklin ML, Day S. Bilateral choroidal excavation in juvenile localized scleroderma. Retin Cases Brief Rep. 2018;12(1):21–3. https://doi.org/10.1097/ICB.0000000000000381.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1136/bjo.2007.116038.

    Article  PubMed  PubMed Central  Google Scholar 

  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. https://doi.org/10.3899/jrheum.161442.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1212/WNL.0000000000003239.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1038/s41433-018-0137-9.

    Article  Google Scholar 

  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. https://doi.org/10.1007/s10067-013-2458-1.

    Article  PubMed  Google Scholar 

  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. https://doi.org/10.1002/acr.21687.

    Article  CAS  Google Scholar 

  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. https://doi.org/10.1111/jdv.14458.

    Article  CAS  PubMed  Google Scholar 

  94. Osminina M, Geppe N, Afonina E. Scleroderma “en coup de sabre” with epilepsy and uveitis successfully treated with tocilizumab. Reumatol Clin. 2018. https://doi.org/10.1016/j.reuma.2018.05.001.

  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).

  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. https://doi.org/10.1093/rheumatology/kex382. Case series of 5 patients with localized scleroderma, demonstrates safety and presumed efficacy of tocilizumab for severe refractory disease.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. NYU Multiple Sclerosis Comprehensive Care Center, Department of Neurology, New York University School of Medicine, New York, NY, USA

    Asya I. Wallach & Ilya Kister

  2. Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA

    Cynthia M. Magro

  3. Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA

    Andrew G. Franks Jr

  4. Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, NY, USA

    Andrew G. Franks Jr

  5. Community Care Rheumatology, Saratoga Springs, NY, USA

    Lee Shapiro

Authors
  1. Asya I. Wallach
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cynthia M. Magro
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Andrew G. Franks Jr
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Lee Shapiro
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Ilya Kister
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Asya I. Wallach.

Ethics declarations

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.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Neurology of Systemic Diseases

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wallach, A.I., Magro, C.M., Franks, A.G. et al. Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma. Curr Neurol Neurosci Rep 19, 11 (2019). https://doi.org/10.1007/s11910-019-0929-8

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11910-019-0929-8

Keywords

Search

Navigation