Investig Magn Reson Imaging.  2018 Sep;22(3):187-193. 10.13104/imri.2018.22.3.187.

Recurrent Neuro-Sweet Disease Associated with Preceding Upper Respiratory Infection: a Case Study

• Suh HB
• Kim HJ

• KMID: 2421552
• DOI: http://doi.org/10.13104/imri.2018.22.3.187

Abstract

Sweet's syndrome also known as acute neutrophilic dermatosis is a multisystem inflammatory disorder characterized by fever, malaise, leukocytosis, and skin lesions. Sweet's syndrome affects multiple organs though only rarely does it affect the central nervous system (CNS) when it does it is called Neuro-Sweet disease (NSD). We report on a case study of a biopsy-proven NSD in a 50 year old man. Serial magnetic resonance imaging (MRI) showed repeated CNS involvement of Sweet's syndrome after a respiratory tract infection preceded it. On the MRI, T2 hyperintense lesions occurred at multiple sites and disappeared after steroid therapy.

Keyword

Sweet's syndrome; Neuro-Sweet disease; Upper respiratory infection; Magnetic resonance imaging

MeSH Terms

Central Nervous System
Fever
Leukocytosis
Magnetic Resonance Imaging
Neutrophils
Respiratory Tract Infections
Skin
Skin Diseases
Sweet Syndrome

Figure

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  Fig. 1 Clinical photograph of the patient showing multiple red erythematous plaques on the face and neck.

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  Fig. 2 Axial FLAIR MR images (a–c) revealed multiple hyperintense lesions in the left frontal lobe, left temporal lobe, left thalamus, pons and midbrain that showed no definite diffusion restriction on DWI (d, e) and no enhancement on contrast-enhanced T1WI (f).

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  Fig. 3 Axial FLAIR images performed 2 years before this presentation (a–d) demonstrated multiple high signal intensity lesions in the left frontal lobe, both temporal lobes, both basal ganglia and insula. The lesions did not show diffusion restriction on DWI (e, f) and contrast enhancement T1WI (g).

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  Fig. 4 Follow up FLAIR images taken after steroid therapy (a–d) showing the extent of multiple hyperintense lesions that diminished in the whole brain.

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  Fig. 5 Photomicrograph of a biopsy specimen showing infiltrations of neutrophils and lymphocytes (Hematoxylin & Eosin, × 400).

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  Fig. 6 Follow up FLAIR images after treatment (a–c) revealed multiple abnormal high signals that had disappeared in the whole brain.

Reference

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

2. Cohen PR, Kurzrock R. Sweet's syndrome revisited: a review of disease concepts. Int J Dermatol. 2003; 42:761–778.

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

4. Cohen PR. Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007; 2:34.

5. Hiari N, Borland C. A 47-year-old man with neuro-Sweet syndrome in association with Crohn's disease: a case report. J Med Case Rep. 2009; 3:8997.

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

7. Singh JS, Costello F, Nadeau J, Witt S, Trotter MJ, Goyal M. Case 176: Neuro-sweet syndrome. Radiology. 2011; 261:989–993.

8. Kim YK, Bang D. Sweet's syndrome associated with bacterial meningitis. Ann Dermatol. 1996; 8:66–69.

9. Akiba C, Esaki T, Ando M, et al. Possible neuro-Sweet disease mimicking brain tumor in the medulla oblongata--case report. Neurol Med Chir (Tokyo). 2011; 51:140–114.

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