ABSTRACT
Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate.
ABSTRACT
BACKGROUND: This report studies the clinical features of Behçet disease (BD) in children and compares our results with other international studies. METHODS: We retrospectively reviewed patient data that included the clinical picture, HLA typing, and treatment in BD cases. RESULTS: This study reviewed data from a total of nine children with BD. Median age at presentation was seven years, with a male to female ratio of 2:1. There was one patient who had Down's syndrome. Oral ulcers were present in all children, while genital ulcers were present in only 66% of cases. Skin manifestation was seen in 88% and uveitis in 55%. There was evidence of gastrointestinal (55%), neurological (55%), and musculoskeletal manifestations (77%). HLA B5 was positive in 66% of cases and 55% had positive family histories. Apart from gastrointestinal symptoms, our results were comparable with other studies. CONCLUSION: Awareness of BD symptoms in the pediatric age group is crucial for early diagnosis and treatment. The coexistence of BD and Down's syndrome needs further genetic study, which may link these two major disorders.
