Acrokeratoelastoidosis: case report

Abstract

Acrokeratoelastoidosis (AKE) is a rare, benign keratoderma that presents as keratotic papules on the borders of palms and soles and frequently involves the dorsum of hands and feet. In most cases it is inherited in an autosomal dominant fashion but also sporadic forms have been reported. Its pathogenesis remains unclear. Histopathology shows  the presence of a reduced number of thick and fragmented elastic fibers in the dermis (elastorhexis).  Various forms of treatments have been attempted with modest or poor results. 

We present an inherited case of AKE in an 14-year-old girl with papular elements simmetrically spread to the knuckles of the fingers from the age of eight.