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Clin Rheumatol (2011) 30:16411645
DOI 10.1007/s10067-011-1830-2
CASE BASED REVIEW
Neonatal Behets disease without maternal history
Yung-Sen Chang & Yao-Hsu Yang & Bor-Luen Chiang
Received: 4 July 2011 /Accepted: 5 August 2011 /Published online: 26 August 2011 # Clinical Rheumatology 2011
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 Behet's disease to date. This is the first reported case in literature of neonatal Behet's disease without a maternal history of Behet's disease, which raises doubts about the assumed role of maternal antibodies in the pathogenesis of neonatal Behet's disease. A literature review of neonatal Behet'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.
Keywords Behet's disease . Gastrointestinal . Maternal history. Neonatal . Pathogenesis
Introduction
Behet's disease (BD) is a chronic multisystem disorder with unknown etiologies. It is uncommon in children and extremely rare in neonates. The previously reported cases
of neonatal BD all had a maternal history of BD, and the mothers were all symptomatic during pregnancy [15]. This suggested that transplacental passage of maternal antibodies might have a role in the pathogenesis of neonatal BD. However, we report a case of neonatal Behet's disease, presenting with recurrent oral and genital ulcers and intestinal ulceration, without maternal history of Behet's disease.
Case report
A female baby was born at 37 weeks gestation with a normal perinatal and birth history. The 38-year-old mother has no known history of systemic or autoimmune diseases. She has a normal 1-year-old child from the same marriage. The neonate presented with frequent vomiting since 10 days old. Low-grade fever around 38C and diarrhea occurred since 22 days old. Progressive leukocytosis with a white blood cell (WBC) count up to 30,820/l (31% neutrophils and 51% lymphocytes) and increasingly high C-reactive protein (CRP) level up...