[Hypogammaglobulinemia of variable expression, Addison's disease, Dubin-Johnson's disease]

A girl aged 12 with hypogammaglobulinaemia is described who developed Addison's disease with adrenal antibodies at the age of eight. This unusual finding is rarely seen in childhood except in the syndrome of juvenile familial polyendocrinopathy and mucocutaneous moniliasis. By chance the child also had Dubin-Johnson syndrome which could alter the metabolism of therapeutically administered drugs and steroids.