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Neuro-Sweet disease

Clinical manifestations and criteria for diagnosis

From the Departments of Neurology and Clinical Research (Drs. Hisanaga and Iwasaki), Miyagi National Hospital, Miyagi, Japan; and the Department of Neurology (D. Itoyama), Tohoku University School of Medicine, Tohoku, Japan.

Address correspondence and reprint requests to Dr. Kinya Hisanaga, Department of Neurology, Miyagi National Hospital, 100 Kassenhara, Takase, Yamamoto, Watari, Miyagi 989-2202, Japan; e-mail: hisanaga{at}mnh.go.jp

Background: Sweet disease, also known as acute febrile neutrophilic dermatosis, is a multisystem inflammatory disorder characterized by painful erythematous plaques and aseptic neutrophilic infiltration of various organs. Skin biopsies typically demonstrate dermal infiltration with neutrophils in the absence of vasculitis. Sweet disease responds to systemic corticosteroids. The CNS can also be involved.

Methods: The authors performed a survey on neuro-Sweet disease (NSD) in Japan and obtained detailed information about 16 cases. They analyzed 42 cases, including 26 cases documented in the literature, and assessed clinical and laboratory criteria for the diagnosis.

Results: Thirteen cases also fulfilled the criteria for the diagnosis of Behçet disease. The clinical features of 27 cases, which the authors classified as probable NSD, are as follows: 1) both sexes are almost evenly affected; 2) people of ages 30 to 70 years are affected; 3) encephalitis and meningitis are common neurologic manifestations; 4) any region of the CNS can be involved, resulting in a variety of neurologic symptoms; 5) there is a strong human leukocyte antigen-Cw1 association; 6) systemic corticosteroids are highly effective for most of the neurologic manifestations, although recurrences are not infrequent.

Conclusions: Neuro-Sweet disease is a distinct entity that may account for some cases of idiopathic encephalomeningitis.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 24 issue to find the title link for this article.

*See the Appendix for a list of Group members.

Supported in part by a grant from Research Committee on Neuroimmunological Diseases, the Ministry of Health, Labor and Welfare, Japan.

Received July 14, 2004. Accepted in final form February 11, 2005.

This article has been cited by other articles:

				Y. Kokubo, S. Kuzuhara, K. Isoda, K. Sato, N. Kawada, and Y. Narita Neuro-Sweet disease: report of the first autopsy case J. Neurol. Neurosurg. Psychiatry, September 1, 2007; 78(9): 997 - 1000. [Abstract] [Full Text] [PDF]