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- Title
A case of neonatal sweet syndrome associated with mevalonate kinase deficiency.
- Authors
Irwin, Margaret; Tanawattanacharoen, Veeraya K.; Turner, Amy; Son, Mary Beth F.; Hale, Rebecca C.; Platt, Craig D.; Putra, Juan; Schmidt, Birgitta A.R.; Wasserman, Mollie G.
- 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.
- Subjects
MEVALONATE kinase; SWEET'S syndrome; PYODERMA gangrenosum; INFLAMMATORY bowel diseases
- Publication
Pediatric Rheumatology, 2023, Vol 21, Issue 1, p1
- ISSN
1546-0096
- Publication type
Case Study
- DOI
10.1186/s12969-023-00887-8