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

A case of Sweet syndrome involving the central nervous system

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초록

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)

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)

참고문헌 (14)

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  2. Mizoguchi M, "Acute febrile neutrophilic dermatosis (Sweet syndrome) in Behcet’ disease", Br J Dermatol, 116, pp.727~734 (1987)
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  4. von den Driesch, "Sweet's syndrome (acute febrile neutrophilic dermatosis)", J Am Acad Dermatol, 31, pp.535~556 (1994)
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  6. Hisanaga K, ""Neuro-sweet disease": benign recurrent encephalitis with neutrophilic dermatosis", Arch Neurol, 56, pp.1010~1013 (1999)
  7. Martinez E, "Sweet's syndrome associated with cerebrospinal fluid neutrophilic pleocytosis", Int J Dermatol, 34, pp.73~74 (1995)
  8. Druschky A, "Sweet's syndrome (acute febrile neutrophilic dermatosis) affecting the central nervous system", J Neurol, 243, pp.556~557 (1996)
  9. Mizoguchi M, "Human leukocyte antigen in Sweet’ syndrome and its relationship to Behcet's disease", Arch Dermatol, 124, pp.1069~1073 (1988)
  10. Yoshiura K, "A case of Sweet’ syndrome with neurological symptoms", Clin Neurol, 29, pp.1081~ (1989)
  11. Noda K, "Sweet's syndrome associated with encephalitis", J Neurol Sci, 188, pp.95~97 (2001)
  12. Komiya I, "Superoxide anion hyperproduction by neutrophils in a case of myelodysplastic syndrome: association with Sweet's syndrome and interstitial pneumonia", Cancer, 67, pp.2337~2341 (1991)
  13. Burrall B, "Sweet's syndrome (acute febrile neutrophilic dermatosis).", Dermatol Online J, 5, pp.8~ (1999)
  14. Cohen PR, "Sweet's syndrome: a review of current treatment options", Am J Clin Dermatol, 3, pp.117~131 (2002)

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