Focal acral hyperkeratosis is characterized by the same clinical appearance as acrokeratoelastoidosis, but without abnormalities in the elastic fibers. We present the case of a woman with a 10-year case of dermatosis localized on the palms, soles and dorsum of the metacarpophalangeal joints, consisting of multiple polygonal papules and associated hyperhydrosis, clinically compatible with acrokeratoelastoidosis. Her father had a history of the disease. In addition, the patient presented with palmoplantar pitted keratolysis. The histopathological study ruled out elastorrhexis, and the pitted keratolysis was corroborated by the clinical appearance and the presence of coccoid elements in the stratum corneum, evident with a PAS stain. In our opinion, the focal acral hyperkeratosis is not a separate entity from the acrokeratoelastoidosis.
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