Abstract
Focal acral hyperkeratosis (FAH) is a rare genodermatosis with an autosomal dominant pattern of inheritance; however, it may also be sporadic. FAH is characterized by late-onset crateriform keratotic papules, some coalescing into plaques, along the borders of the hands and feet. We herein report a case of FAH in a 47-year-old male with a family history of similar lesions in three generations. The histological findings revealed focal areas of orthohyperkeratosis over an area of depressed but otherwise normal epidermis. The dermis showed no specific changes, which distinguished this case from acrokeratoelastoidosis, which shows elastorrhexis of clinically similar lesions.