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53 - SWEET’S SYNDROME (ACUTE FEBRILE NEUTROPHILIC DERMATOSIS)

from PART V: - SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM

Published online by Cambridge University Press:  06 January 2010

Louis R. Caplan
Affiliation:
Beth Israel Deaconess Medical Center, Boston
Julien Bogousslavsky
Affiliation:
Valmont Clinique, Glion, Switzerland
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Summary

Sweet's syndrome (SS), also referred to as acute febrile neutrophilic dermatosis, is characterized by a constellation of symptoms and findings: fever, neutrophilia, erythematous and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, and prompt improvement of both symptoms and lesions after the initiation of treatment with systemic corticosteroids. Many instances are related to lympho proliferative disorders such as acute and chronic leukemia, acute and chronic lymphatic leukemia, hairy cell leukemia, polycythemia vera, non-Hodgkin's lymphoma, Hodgkin's lymphoma, and other diseases of the hematopoietic system. Encephalitis and meningitis were the most common neurological manifestations. An elevated erythrocyte sedimentation rate (ESR) and peripheral leukocytosis with neutrophilia are the most consistent laboratory findings in SS. The standard therapy is administration of prednisone or prednisolone at an initial dose of 0.5-1.5 mg/kg body weight with a subsequent slow reduction over 2-4 weeks.
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Publisher: Cambridge University Press
Print publication year: 2008

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