A Case of Neonatal Sweet Syndrome Associated with Mevalonate Kinase Deficiency

Abstract

Background: Sweet Syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with SS. Case Presentation: Through a multi-disciplinary approach, this patient was also found to have very-early onset inflammatory bowel disease (VEO-IBD) and Mevalonate kinase-associated disease (MKAD). While prior case studies have characterized an association between VEO-IBD and MKAD, there is no literature describing the association of all three diagnoses this case: SS, VEO-IBD and MKAD. Initiation of canakinub in this patient resulted in successful symptomatic control. Conclusions: This case highlights the importance of a multi-disciplinary approach to rare diagnoses, and collaboration during cases with significant diagnostic uncertainty.