Dataset Information
A case of neonatal sweet syndrome associated with mevalonate kinase deficiency.
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ABSTRACT: Background
Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopathologic variant of SS. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with H-SS.Case presentation
Through a multidisciplinary 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: H-SS, VEO-IBD and MKAD. Initiation of canakinumab in this patient resulted in successful control of the disease.Conclusions
This case highlights the importance of a multidisciplinary approach to rare diagnoses, and collaboration during cases with significant diagnostic uncertainty.
SUBMITTER: Irwin M
PROVIDER: S-EPMC10496215 | biostudies-literature | 2023 Sep
REPOSITORIES: biostudies-literature
Publications
A case of neonatal sweet syndrome associated with mevalonate kinase deficiency.
Pediatric rheumatology online journal 20230912 1
<h4>Background</h4>Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopathologic variant of SS. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with H-SS.<h4>Case presentation</h4>Through a multidisciplinary approach, this patient was also found to have very early onset inflammatory bowel disease ...[more]