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Published on: September 20, 2018
Neonatal Behçet's syndrome: a case report and literature review
Sandra Catarina Lino Ferreira Ferraz1, David Rabiço-Costa2, António Andrade3
1Department of Pediatrics, Local Health Unit of Alto Minho, Viana do Castelo.
None:
Behçet's syndrome is a rare, chronic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and eye inflammation. While most children born to mothers with Behçet's syndrome remain asymptomatic, some newborns may develop symptoms due to unclear pathological mechanisms. A male newborn, born at 39 weeks to a mother with a 14-year history of Behçet's syndrome, developed oral lesions on the 10th day of life. The lesions progressed, becoming ulcerated and causing painful feeding. Additional findings included a few facial pustules, pathergy, leukocytosis, and elevated inflammatory markers. Despite multiple therapies, the condition persisted. A biopsy of the tongue lesion was performed, and a diagnosis of neonatal Behçet's syndrome was made based on the clinical course, negative microbiological studies, and maternal history. The patient was treated with intravenous methylprednisolone, followed by oral steroids, with resolution within 3 months. Neonatal Behçet's syndrome is a very rare condition, with only a few cases reported in the literature. Most cases have mild clinical presentations, resolving spontaneously or with corticosteroids. However, severe cases with airway obstruction or neurological complications have been reported. This case demonstrates a favorable and self-limiting course. The underlying mechanisms causing symptoms in newborns of mothers with Behçet's syndrome remain unclear. Reporting these rare cases can help identify risk factors and improve early diagnosis.

