WHEN A SWEET RASH LEADS TO A SOUR DIAGNOSIS

Abstract

<h3>Introduction</h3> Sweet syndrome (SS) is a rare neutrophilic dermatosis with symptoms including fever, neutrophilia and tender erythematous skin lesions. Often preceded by upper respiratory infections, SS classically presents in middle-aged women. Pediatric SS is rare; SS as the presenting feature of chronic granulomatous disease (CGD) has only been described in two case reports previously. We describe a patient presenting with SS, who was found to have CGD. <h3>Case Description</h3> A 12 month-old male presented for evaluation of persistent fevers, lymphadenopathy and pustular rash. It was thought that he had an atypical presentation of Kawasaki Disease, and he was treated with IVIG in addition to doxycycline for empiric coverage of possible infection. Biopsy of the rash confirmed neutrophilic dermatitis, consistent with SS. One week later, he was readmitted for recurrence of fever, anemia, transaminitis and elevated inflammatory markers. Genetic testing for autoinflammatory disorders was completed, and a pathogenic hemizygous mutation in <i>CYBB</i> was found. This prompted dihydrohodamine testing which confirmed abnormal neutrophil respiratory burst. His rash resolved after treatment with corticosteroids which is the mainstay treatment for SS. He was started on antimicrobial prophylaxis and referred for evaluation for hematopoietic stem cell transplant. <h3>Discussion</h3> In children, SS is associated with chronic inflammatory conditions, viral illnesses or malignancy. Immunodeficiency and lupus are also associated with SS, specifically in neonates. This case highlights that immunodeficiency should be considered in cases of SS even in older children. Immunologists play a role in the evaluation of these children.