A case of neonatal sweet syndrome associated with mevalonate kinase deficiency.
Pediatr Rheumatol Online J
; 21(1): 101, 2023 Sep 12.
Article
em En
| MEDLINE
| ID: mdl-37700301
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.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doenças Inflamatórias Intestinais
/
Síndrome de Sweet
/
Deficiência de Mevalonato Quinase
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Risk_factors_studies
Limite:
Adult
/
Humans
/
Newborn
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article