Acrokeratoelastoidosis: a spectrum of diseases

Two familial cases of acrokeratoelastoidosis (AKE) are reported. As a quite uncommon feature, both showed a distinct violaceous hue in addition to the characteristic clinicopathological changes. Furthermore a case of keratoelastoidosis marginalis of the hands, also showing involvement of the feet, is described. This latter syndrome may cause confusion with AKE, but appears to be a separate entity. The classification of AKE is discussed. It is suggested that AKE is a syndrome with a broad spectrum of clinical and histological abnormalities.