Acrokeratoelastoidosis: report of a case

Acrokeratoelastoidosis (AKE) is a rare chronic skin disorder characterized by firm papules or plaques on the sides of the hands and feet. It appears to be a genodermatosis with an autosomal dominant inheritance pattern and a possible linkage to chromosome 2. However, many sporadic reports of acquired cases seen in older individuals with a history of both excessive sun exposure and chronic trauma of the hands have also appeared in literature. Although poorly understood, lesions may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. We describe the case of a 42-year-old woman and review the patient's clinical and histopathologic features, the causes of the disease as well as the differential diagnosis made.