Leopard syndrome

The leopard, or multiple lentigens syndrome includes various congenital malformations affecting the skin, cardiovascular system, inner ear, genitalia and skeleton. It is an autosomal dominant syndrome with variable penetrance. We describe two children who showed the characteristic skin features, facial appearance and cardiac (including electrocardiographic) abnormalities. In both patients the diagnosis was made at a relatively young age. Multiple lentigens are an important physical feature and their presence should arouse suspicion of possible multiple system involvement.