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Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome

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Abstract

Sweet’s syndrome (SS) is an inflammatory disease characterized by fever, leucocytosis and distinctive skin lesions that histologically consist of a dermal infiltrate of neutrophils with nuclear fragmentation. Aseptic neutrophilic inflammation may occur also in other organs. Central nervous system involvement in SS, Neuro-Sweet’s syndrome (NSS), is rare and reported especially among Asian patients. A systematic review of the literature has been performed to find articles reporting cases of SS with neurological involvement. The search terms: “Sweet’s syndrome/disease with neurological involvement, Neuro Sweet Syndrome/Disease” were used in the Pubmed Database. Sixty-nine NSS patients including 46 males and 23 females, more Asian than Caucasian, have been described from 1983 to date. The average age was 48.7 year-old. The most representative neurologic symptom was the altered state of consciousness, followed by headache and memory disorders. Differently from SS with skin or other district involvement, NSS appears to be more common in Asian patients than in Caucasian ones and affects mainly the male sex in the third or fourth decade of life. A very wide range of symptoms and signs can occur, depending on which part of the nervous system is affected. Initial presentation is usually with the SS typical skin lesions followed by neurological involvement. However, also an opposite presentation or a simultaneous skin and nervous involvement may happen. Awareness of the possible neurological complications in SS is important to avoid unnecessary therapies for other forms of meningoencephalitis and lead to successful treatment with systemic corticosteroids.

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References

  1. Sweet RD (1964) An acute febrile neutrophilic dermatosis. Br J Dermatol 76:349–356

    Article  CAS  PubMed  Google Scholar 

  2. Cohen PR (2007) Sweet’s syndrome–a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2:34

    Article  PubMed  PubMed Central  Google Scholar 

  3. Hönigsmann H, Kempter R, Wolff K (1979) Acute febrile neurophilic dermatosis (Sweet’s syndrome) report of two cases. Wien Klin Wochenschr 91:842

    PubMed  Google Scholar 

  4. Ratzinger G, Burgdorf W, Zelger BG, Zelger B (2007) Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. Am J Dermatopathol 29:125–133

    Article  PubMed  Google Scholar 

  5. Malone JC, Slone SP, Wills-Frank LA, Fearneyhough PK, Lear SC, Goldsmith LJ, Hood AF, Callen JP (2002) Vascular inflammation (vasculitis) in sweet syndrome: a clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol 138:345–349

    Article  PubMed  Google Scholar 

  6. Elsner P, Hartmann AA, Lechner W (1986) Sweet’s syndrome associated with Yersinia enterocolitica infection. Dermatologica 173:85–89

    Article  CAS  PubMed  Google Scholar 

  7. Escallier F, Gaudard S, Courtois JM, Dalac S, Collet E, Lambert D (1990) Sweet’s syndrome and Yersinia enterocolitica infection. Ann Dermatol Venereol 117:858–860

    CAS  PubMed  Google Scholar 

  8. Rochet NM, Chavan RN, Cappel MA, Wada DA, Gibson LE (2013) Sweet syndrome: clinical presentation, associations, and response to treatment in 77 patients. J Am Acad Dermatol 69:557–564

    Article  CAS  PubMed  Google Scholar 

  9. Going JJ (1987) Is the pathogenesis of Sweet’s syndrome mediated by interleukin-1? Br J Dermatol 116:282–283

    Article  CAS  PubMed  Google Scholar 

  10. Giasuddin AS, El-Orfi AH, Ziu MM, El-Barnawi NY (1998) Sweet's syndrome: is the pathogenesis mediated by helper T cell type 1 cytokines? J Am Acad Dermatol 39:940–943

    Article  CAS  PubMed  Google Scholar 

  11. Hisanaga K, Hosokawa M, Sato N, Mochizuki H, Itoyama Y, Iwasaki Y (1999) “Neuro-sweet disease”: benign recurrent encephalitis with neutrophilic dermatosis. Arch Neurol 56:1010–1013

    Article  CAS  PubMed  Google Scholar 

  12. Mizoguchi M, Matsuki K, Mochizuki M et al (1988) Human leukocyte antigen in Sweet’s syndrome and its relationship to Behçet’s disease. Arch Dermatol 124:1069–1073

    Article  CAS  PubMed  Google Scholar 

  13. Hisanaga K (2007) Neuro-neutrophilic disease: neuro-Behçet disease and neuro-Sweet disease. Intern Med 46:153–154

    Article  PubMed  Google Scholar 

  14. Singh JS, Costello F, Nadeau J, Witt S, Trotter MJ, Goyal M (2011) Case 176: neuro-sweet syndrome. Radiology 261:989–993

    Article  PubMed  Google Scholar 

  15. Chiba S (1983) Sweet’s syndrome with neurologic signs and psychiatric symptoms. Arch Neurol 40:829

    Article  CAS  PubMed  Google Scholar 

  16. Furukawa F, Toriyama R, Kawanishi T (1992) Neutrophils in cerebrospinal fluid of a patient with acute febrile neutrophilic dermatosis (Sweet’s syndrome). Int J Dermatol 31:670–671

    Article  CAS  PubMed  Google Scholar 

  17. Dunn TR, Saperstein HW, Biederman A, Kaplan RP (1992) Sweet syndrome in a neonate with aseptic meningitis. Pediatr Dermatol 9:288–292

    Article  CAS  PubMed  Google Scholar 

  18. Martínez E, Fernández A, Mayo J, Manrique P, Collazos J (1995) Sweet’s syndrome associated with cerebrospinal fluid neutrophilic pleocytosis. Int J Dermatol 34:73–74

    Article  PubMed  Google Scholar 

  19. Uysal H, Vahaboğlu H, Inan L, Vahaboğlu G (1993) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in neuro-Behçet’s disease. Clin Neurol Neurosurg 95:319–322

    Article  CAS  PubMed  Google Scholar 

  20. Druschky A, von den Driesch P, Anders M, Claus D, Neundörfer B (1996) Sweet’s syndrome (acute febrile neutrophilic dermatosis) affecting the central nervous system. J Neurol 243:556–557

    Article  CAS  PubMed  Google Scholar 

  21. Ohori N, Kinoshita T, Toda K, Ohta S, Yoshimura T (1999) A case of Sweet’s syndrome (acute febrile neutrophilic dermatosis) showing transient jargon aphasia. Rinsho Shinkeigaku 39:1156–1159

    CAS  PubMed  Google Scholar 

  22. Pharis DB, Cerenko D, Caughman SW (2000) Sweet’s syndrome in a patient with idiopathic progressive bilateral sensorineural hearing loss. J Am Acad Dermatol 42(5 Pt 2):932–935

    Article  CAS  PubMed  Google Scholar 

  23. Noda K, Okuma Y, Fukae J et al (2001) Sweet’s syndrome associated with encephalitis. J Neurol Sci 188:95–97

    Article  CAS  PubMed  Google Scholar 

  24. Kato T, Kunikata N, Taira H, Kobayashi N, Tanji K, Endo M (2002) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) with nodular episcleritis and polyneuropathy. Int J Dermatol 41(2):107–109

    Article  PubMed  Google Scholar 

  25. Cano A, Ribes R, de la Riva A, Rubio FL, Sánchez C, Sancho JL (2002) Idiopathic hypertrophic cranial pachymeningitis associated with Sweet’s syndrome. Eur J Radiol 44:139–142

    Article  PubMed  Google Scholar 

  26. Nobeyama Y, Kamide R (2003) Sweet’s syndrome with neurologic manifestation: case report and literature review. Int J Dermatol 42:438–443

    Article  PubMed  Google Scholar 

  27. Stenzel W, Frosch PJ, Schwarz M (2003) Sweet’s syndrome associated with acute benign encephalitis. A drug induced aetiology. J Neurol 250:770–771

    Article  CAS  PubMed  Google Scholar 

  28. Hisanaga K, Iwasaki Y, Itoyama Y, Neuro-Sweet Disease Study Group (2005) Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology 64:1756–1761

    Article  PubMed  Google Scholar 

  29. Tsunemi T, Sakai Y, Tsunoda K, Irie Y, Wada Y (2006) Neuro-Behçets/neuro-Sweets disease presents simultaneously with severe tonsillitis, and features mimicking bacterial meningitis with skin lesions. Intern Med 45:1315–1317

    Article  PubMed  Google Scholar 

  30. Song IU, Kim JS, Kim YI, Lee KS (2007) Sweet’s syndrome associated with acute-onset encephalitis. Eur Neurol 57:182–184

    Article  PubMed  Google Scholar 

  31. Kokubo Y, Kuzuhara S, Isoda K, Sato K, Kawada N, Narita Y (2007) Neuro-Sweet disease: report of the first autopsy case. J Neurol Neurosurg Psychiatry 78:997–1000

    Article  PubMed  PubMed Central  Google Scholar 

  32. Fukae J, Noda K, Fujishima K et al (2007) Successful treatment of relapsing neuro-Sweet’s disease with corticosteroid and dapsone combination therapy. Clin Neurol Neurosurg 109:910–913

    Article  PubMed  Google Scholar 

  33. Kimura A, Sakurai T, Koumura A et al (2008) Longitudinal analysis of cytokines and chemokines in the cerebrospinal fluid of a patient with Neuro-Sweet disease presenting with recurrent encephalomeningitis. Intern Med 47:135–141

    Article  PubMed  Google Scholar 

  34. Aydemir H, Oztoprak N, Celebi G et al (2008) Pulmonary and central nervous system involvement in Sweet’s syndrome: a very rare case report. Intern Med 47:1481–1484

    Article  PubMed  Google Scholar 

  35. Fukushima K, Hineno A, Kodaira M et al (2008) Reversible extensive leukoencephalopathy in Sweet disease: a case report. J Neurol Sci 275:178–180. doi:10.1016/j.jns.2008.08.006

    Article  PubMed  Google Scholar 

  36. Sobol UA, Sherman KL, Smith J et al (2009) Sweet’s syndrome with neurologic manifestations in a patient with esophageal adenocarcinoma: case report and review of the literature. Int J Dermatol 48:1062–1065

    Article  PubMed  Google Scholar 

  37. Sato M, Yoneda M, Kumakiri M, Kuriyama M (2009) Case of neuro-Behçet disease with HLA-B54, Cw1, which is difficult in clinical diagnosis from neuro-Sweet disease. Rinsho Shinkeigaku 49:479–482

    Article  PubMed  Google Scholar 

  38. Watanabe T, Nakashima K, Shindo M, Yoshida Y, Yamamoto O (2009) Multiorgan involvement in Sweet’s syndrome. Clin Exp Dermatol 34:e343–e344. doi:10.1111/j.1365-2230.2009.03290.x

    Article  CAS  PubMed  Google Scholar 

  39. Hiari N, Borland C (2009) A 47-year-old man with neuro-Sweet syndrome in association with Crohn’s disease: a case report. J Med Case Rep 3:8997. doi:10.4076/1752-1947-3-8997

    Article  PubMed  PubMed Central  Google Scholar 

  40. Shibata K, Tateishi T, Yamasaki R, Ohyagi Y, Kira J (2010) Successful treatment of a case of steroid-dependent neuro-Sweet disease with dapsone. Rinsho Shinkeigaku 50:257–261

    Article  PubMed  Google Scholar 

  41. Niwa F, Tokuda T, Kimura M, Azuma Y, Mizuno T, Nakagawa M (2010) Self-remitting and reversible parkinsonism associated with neuro-Sweet disease. Intern Med 49:1201–1204

    Article  PubMed  Google Scholar 

  42. Mariën P, Tops W, Crols R, Jonkers R, De Deyn PP, Verhoeven J (2012) Grammar disruption in a patient with Neuro-Sweet syndrome. Neurocase 18:235–247

    Article  PubMed  Google Scholar 

  43. Akiba C, Esaki T, Ando M et al (2011) Possible neuro-Sweet disease mimicking brain tumor in the medulla oblongata––case report. Neurol Med Chir (Tokyo) 51:140–143

    Article  Google Scholar 

  44. Ohe Y, Nakazato Y, Ishizawa K, Deguchi I, Tamura N, Araki N (2011) A case of neuro-neutrophilic disease presenting with 5 months’ with cognitive decline, meningoencephalitis, and marked systemic inflammatory findings, and diagnosed with brain biopsy. Rinsho Shinkeigaku 51:21–26

    Article  PubMed  Google Scholar 

  45. Lobo AM, Stacy R, Cestari D, Stone JH, Jakobiec FA, Sobrin L (2011) Optic nerve involvement with panuveitis in Sweet syndrome. Ocul Immunol Inflamm 19:167–170

    Article  PubMed  Google Scholar 

  46. Shimizu Y, Watanabe T, Nakada M et al (2012) Neuro-Sweet disease mimicking recurrent anaplastic astrocytoma associated with therapy-related myelodysplastic syndrome: a case report. Clin Neurol Neurosurg 114:1049–1051

    Article  PubMed  Google Scholar 

  47. Maxwell G, Archibald N, Turnbull D (2012) Neuro-Sweet’s disease. Pract Neurol 12:126–130

    Article  PubMed  Google Scholar 

  48. Makimoto G, Manabe Y, Yamakawa C et al (2012) Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation. Neurol Int 4:e5

    Article  PubMed  PubMed Central  Google Scholar 

  49. Drago F, Ribizzi G, Ciccarese G, Agnoletti AF, Parodi A (2014) Recurrent episodes of neuro-Sweet syndrome in a Caucasian patient. J Am Acad Dermatol 71:192–193

    Article  PubMed  Google Scholar 

  50. Koay CL, Chew FL, Chong KY, Subrayan V (2013) Compressive optic neuropathy: a unique presentation of Sweet syndrome. Indian J Ophthalmol 61:140–141

    Article  PubMed  PubMed Central  Google Scholar 

  51. Nukui T, Takashima S, Taguchi Y, Dougu N, Konishi H, Tanaka K (2014) A case of neuro-Sweet disease showing the close association between disease activity and levels of soluble IL-2 receptor. Rinsho Shinkeigaku 54:876–881

    Article  PubMed  Google Scholar 

  52. Nakatani R, Naba I, Kawasaki Y, Moriya M, Nakano M, Tatsumi C (2014) A case of possible neuro-Sweet disease with prolonged disturbance of consciousness and no dermal lesion during the course of dementia. Rinsho Shinkeigaku 54:130–134

    Article  PubMed  Google Scholar 

  53. Nakanishi E, Sawamura M, Maruhama S, Yamada H, Kim G, Harada K (2015) Neuro-neutrophilic disease suspected by human leukocyte antigen (HLA) typing and brain biopsy: a case report. Rinsho Shinkeigaku 55:13–17

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. DISSAL-Section of Dermatology-IRCCS Azienda Universitaria Ospedaliera San Martino-IST, Largo Rosanna Benzi 10, 16132, Genoa, Italy

    Francesco Drago, Giulia Ciccarese, Arianna Fay Agnoletti & Aurora Parodi

  2. Pathology Unit, IRCCS Azienda Universitaria Ospedaliera San Martino-IST, 16132, Genoa, Italy

    Francesca Sarocchi

Authors
  1. Francesco Drago
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  2. Giulia Ciccarese
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  3. Arianna Fay Agnoletti
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  4. Francesca Sarocchi
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  5. Aurora Parodi
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Correspondence to Giulia Ciccarese.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or National Research Committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Drago, F., Ciccarese, G., Agnoletti, A.F. et al. Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome. Acta Neurol Belg 117, 33–42 (2017). https://doi.org/10.1007/s13760-016-0695-1

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  • DOI: https://doi.org/10.1007/s13760-016-0695-1

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