A case of Sweet syndrome involving the central nervous system

Hyun-Young Jung,; Soo-Kyoung Kim,; Keun-Woo Park,; Cheol-Yeon Park,; Seung-Hie Chung,; Sung-Hoon Park,; Jung-Yoon Choe,

Korean J Med > Volume 75(4); 2008 > Article

Original Article

The Korean Journal of Medicine 2008;75(4):463-466.

A case of Sweet syndrome involving the central nervous system

Hyun-Young Jung, Soo-Kyoung Kim, Keun-Woo Park, Cheol-Yeon Park, Seung-Hie Chung, Sung-Hoon Park, Jung-Yoon Choe

경희대학교 의과대학 순환기내과학교실

증례: 중추신경계 침범을 보인 Sweet 증후군 1예: Neuro-Sweet disease

정현영 . 김수경 . 박근우 . 박철연 . 정승혜 . 박성훈 . 최정윤, Soo-Kyoung Kim, Keun-Woo Park, Cheol-Yeon Park, Seung-Hie Chung, Sung-Hoon Park, Jung-Yoon Choe

Abstract

Sweet syndrome is an unusual disease characterized by the sudden onset of fever, leukocytosis, and painful erythematous plaques, and the dermal infiltration of neutrophils at the site of skin lesions. Although Sweet syndrome can also present with extra-cutaneous manifestations, involvement of the central nervous system (CNS) is rarely reported. We describe a case of Sweet syndrome involving the CNS in a 46-year-old male with a disturbance of consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid examination disclosed neutrophilic pleocytosis without decreased glucose and protein levels. HLA typing showed B54, which is frequently seen in Sweet syndrome. Brain magnetic resonance imaging showed abnormal signal intensity lesions in the left temporal lobe. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet syndrome. The confused mentality, fever, and erythematous skin plaques resolved after the administration of systemic corticosteroids. (Korean J Med 75:463-466, 2008)

Key Words: Sweet syndrome; Central nervous system