title: Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin.
abstract: Painful erythematous plaques and a pathognomonic, dense, dermal infiltrate of neutrophils characterize Sweet syndrome. Although numerous instances of this disease have been reported in children, only four have had an associated immunodeficiency to date. Sweet syndrome has been traditionally treated with systemic corticosteroids despite an unknown pathogenesis. Prior reports suggest that immunodeficient children with Sweet syndrome may have a persistent course and fail to respond to standard treatment. We describe a child with debilitating Sweet syndrome and a primary immunodeficiency whose symptoms were controlled with intravenous immunoglobulin and dapsone.
authors.affiliation: Department of Pediatrics, North Carolina Children's Hospital, University of North Carolina Chapel Hill, North Carolina, USA. e_haliasos@hotmail.com
authors.forename: Elena
authors.initials: E
authors.lastname: Haliasos
authors.forename: Bradley
authors.initials: B
authors.lastname: Soder
authors.forename: David S
authors.initials: DS
authors.lastname: Rubenstein
authors.forename: William
authors.initials: W
authors.lastname: Henderson
authors.forename: Dean S
authors.initials: DS
authors.lastname: Morrell
mesh_terms: D002675:Child, Preschool; D003622:Dapsone / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D018570:Risk Assessment; D012720:Severity of Illness Index; D016463:Sweet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D003622:Dapsone