Inherited Heart Conduction Disorders with Skin and Hair Hypopigmentation

This report describes a family with several male members having hypopigmentation in some areas of their skin and hair, and right bundle branch block on the electrocardiogram.Gradually, in a few of them, the conduction abnormalities evolve into a complete atrioventricular block requiring pacemaker implantation.The family pedigree shows an X-linked pattern of transmission of this combination of clinical abnormalities.