Acrokeratoelastoidosis: presentation of a localized lesions to dorsae of hands and feet

Acrokeratoelastoidosis is a rare genodermatosis that presents clinically as shiny keratotic papules and paques along the junctionn of palms or soles with the dorsae of hands or feet. AKE commonly starts at puberty. Here, we present a case of AKE in a 50-year-old Bangladeshi woman with asymptomatic skin lesion on the dorsal surface of the feet and hands since childhood. She denies any family history of similar condition and her parents are not consanguineous.
Skin examination showed multiple keratotic plaques over the dorsae of hands and feet, some of them are in linear configuration. Histological examination of a 4mm punch skin biopsy specimen revealed orthohyperkeratosis, psoriasiform acanthosis and elastotic plaques with elastorrhexis in the dermis. Elastin stain showed increased elastin fragments with elastorrhexis. On the basis of the above clinicopathological findings, a diagnosis of acrokeratoelastoidosis was made. The patient was reassured.