Congenital immunodeficiency in hyper IgE syndrome is characterised by a mar
kedly raised IgE level, recurrent staphylococcal skin infection and pneumat
oceles. Standard treatments include anti-staphylococcal antibiotics. We rep
ort a severely affected patient in whom successful bone marrow transplantat
ion was followed by reappearance of the immunodeficiency. We conclude that
bone marrow transplantation does not cure the immunological features of the
hyper IgE syndrome.