COEXISTENCE OF DUBOWITZ AND HYPER-IGE SYNDROMES - A CASE-REPORT

A case of a 5-year-old girl is described whose clinical features inclu ded postnatal growth retardation, microcephaly and characteristic faci al appearance. These are recognized as the main features of the Dubowi tz syndrome. Apart from these features, our patient had recurrent infe ctions of the sinopulmonary tract, high serum IgE levels, defective ch emotaxis of polymorphonu-clear cells and defective antibody re sponse, findings characterizing the hyper-IgE syndrome. The co-existence of t hese two syndromes is rare and we suggest that this is the first such case in the literature. Conclusion Patients with the Dubowitz syndrome will require Long-term follow up because there is a considerable risk for the syndrome drome to co-exist with primary immunodeficiency or f or malignancies develop.