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Clinical Rheumatology 2011-Dec

Neonatal Behçet's disease without maternal history.

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Yung-Sen Chang
Yao-Hsu Yang
Bor-Luen Chiang

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Abstract

A neonate presented with frequent vomiting since 10 days old, followed by severe diarrhea. Multiple oral ulcers and recurrent genital ulcers subsequently appeared. Colonoscopy showed multiple shallow round ulcerations in the colon. The symptoms responded well to a short course of oral prednisolone. There was a brief recurrence of vomiting, diarrhea, and oral ulcers at 2 months old. The mother has never had any symptoms of Behçet's disease to date. This is the first reported case in literature of neonatal Behçet's disease without a maternal history of Behçet's disease, which raises doubts about the assumed role of maternal antibodies in the pathogenesis of neonatal Behçet's disease. A literature review of neonatal Behçet's disease shows that oral ulcers, skin lesions, fever, and leukocytosis are common features. However, only half of the patients fulfill the classical diagnostic criteria based on adult studies. A treatment consensus for neonatal cases is also lacking.

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