Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin.
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.
Haliasos
Elena
E
Department of Pediatrics, North Carolina Children's Hospital, University of North Carolina Chapel Hill, North Carolina, USA. e_haliasos@hotmail.com
Soder
Bradley
B
Rubenstein
David S
DS
Henderson
William
W
Morrell
Dean S
DS
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
D016756:Immunoglobulins, Intravenous; D003622:Dapsone