Sweet syndrome (
acute, febrile, neutrophilic dermatosis) is characterized by the acute onset of an eruption of painful nodules or erythematous or violaceous plaques on the limbs, face and neck. These symptoms are accompanied by
fever. The diagnostic features include histopathological findings of dermal neutrophilic infiltration without
leukocytoclastic vasculitis or peripheral blood
leukocytosis.
Sweet syndrome is associated with
infection,
malignancies,
autoimmune disease, pregnancy, and drugs. Patients with
Sweet syndrome demonstrate a complete and rapid response to systemic
steroid administration. Recently, a distinct variant of
Sweet syndrome was reported, termed "histiocytoid
Sweet syndrome", in which the infiltration of
myeloperoxidase-positive histiocytoid mononuclear cells are observed (in contrast to the infiltration of neutrophils). The other clinical features are similar to those of classic
Sweet syndrome. Pediatric
Sweet syndrome is uncommon, and the histiocytoid type is even rarer. To date, four cases of histiocytoid
Sweet syndrome have been reported in children. Herein, we describe a case of histiocytoid
Sweet syndrome in an otherwise healthy 10-year-old boy with no underlying systemic disease in whom non-steroidal, anti-inflammatory
drug treatment was successful.