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 Beh et 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.