Neuro-Sweet disease: clinical manifestations and criteria for diagnosis.
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 Behcet 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.
Hisanaga
Kinya
K
Department of Neurology, Miyagi National Hospital, Watari, Miyagi, Japan. hisanaga@mnh.go.jp
Iwasaki
Yuzo
Y
Itoyama
Yasuto
Y
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017668:Age of Onset; D001528:Behcet Syndrome / Q000175:diagnosis / Q000453:epidemiology; D001706:Biopsy; D002490:Central Nervous System / Q000473:pathology / Q000503:physiopathology*; D015897:Comorbidity; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D005260:Female; D006680:HLA Antigens / Q000097:blood; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008581:Meningitis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D008590:Meningoencephalitis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D008875:Middle Aged; D009369:Neoplasms / Q000175:diagnosis / Q000453:epidemiology*; D017678:Sex Distribution; D012867:Skin / Q000473:pathology; D016463:Sweet Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*
D000305:Adrenal Cortex Hormones; D006680:HLA Antigens