Neuro-Sweet's Syndrome with Central Nervous System Involvement / 대한피부과학회지

Sweet's syndrome is an acute reactive condition that is characterized by fever, neutrophilic leukocytosis, erythematous tender edematous plaques or papules, and a dense dermal infiltrate of mature neutrophils. Although the extracutaneous manifestations of Sweet's syndrome are commonly seen in the joints and eyes, the central nervous system is rarely involved. A novel concept designated "Neuro-Sweet's syndrome" has recently been proposed for those cases of Sweet's syndrome that involve the central nervous system. It is essential to evaluate the skin eruptions in patients with recurrent meningoencephalitis of an unknown origin in order to avoid unnecessary empiric therapy. Herein, we report on two men who suffered from acute recurrent meningoencephalitis and they were subsequently diagnosed as having Neuro-Sweet's syndrome.

A Case with Neuro-Sweet Syndrome

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is an unusual disease characterized by fever, leukocytosis, and distinctive skin lesions. Common complications include arthralgia, arthritis, conjunctivitis, and iridocyclitis. However, the involvement of the central nervous system in this disease, termed `neuro-Sweet syndrome' is rarely reported. We present a patient with recurrent encephalitis for 15 years, accompanied with neutrophilic dermatosis, and characterized by HLA-B54 and Cw1, with good responsiveness to corticosteroid.