"Neuro-sweet disease" - Benign recurrent encephalitis with neutrophilic dermatosis

Objective: To describe benign recurrent encephalitis in a case of Sweet syn drome that also showed clinical features of Behcet disease. Case Report: A 37-year-old Japanese man developed relapsing and remitting e ncephalitis and mucocutaneous symptoms mimicking Behcet disease. Magnetic r esonance images showed at least 5 episodes of transient abnormal signal int ensity in various cerebral regions over a period of 5 years. A skin biopsy specimen of the cutaneous edematous erythematous plaques revealed neutrophi lic dermatitis compatible with Sweet syndrome. HLA typing showed B54, which is frequent in Sweet syndrome but rare in Behcet disease. Oral prednisolon e therapy (10-60 mg/d) was remarkably effective for the encephalitis as wel l as for the mucocutaneous symptoms, Conclusion: We propose that there is an entity that is like Sweet disease, but with recurrent encephalitis characterized by an association with HLA-B5 4 and a high responsiveness to corticosteroid therapy, which we have tentat ively named neuro-Sweet disease, that is distinct from the classic central nervous system involvement of Behcet disease.