Acrokeratoelastoidosis

Acrokeratoelastoidosis (AKE) of Oswaldo Costa, or , is a rare autosomal-dominant genodermatosis first described in 1952 by Oswaldo Costa, a Brazilian dermatologist. It characteristically presents with flesh-colored papules on the lateral areas of the palms, soles, and dorsum of hands. Acrokeratoelastoidosis is a type of marginal keratoderma that principally affects the lateral portion of the palmoplantar regions . Apart from inherited cases, there are reports of sporadic occurrences of this condition. The histological features are hyalinized and homogenous collagen, hyperkeratosis,  and a decrease in, and fragmentation of the elastic fibers (elastorrhexis). This rare form of focal acral keratoderma of unknown cause typically begins during childhood, although onset may delay until adolescence. As per the limited published literature on this condition, there seems to be no gender or racial predilection. Another reason for some patients seeking a dermatologist's opinion a few years after the onset is the asymptomatic nature of the lesions. The condition requires differentiation from two sets of disorders: 1) other marginal and focal acral keratodermas, and 2) distinct disorders such as acrokeratosis verruciformis of Hopf. Histopathological findings aid in differentiation between acrokeratoelastoidosis and other clinical simulators. There is no reported morbidity, and apart from cosmetic concerns of some patients, the overall prognosis of AKE is good.