Acrokeratoelastoidosis (AKE) is a rare disease that manifests as wartlike papules along the dorsal palmar junction. It is characterized by orthohyperkeratosis in the horny layer and elastorrhexis in the reticular dermis. Both sporadic and familial cases following autosomal dominant inheritance have been reported. Currently, no effective treatments exist for AKE, which can have a significant cosmetic impact. Here we present the second reported case of unilateral AKE in a 5-year-old African American girl and hypothesize that the mechanism for the unilateral nature of AKE in this patient is genetic mosaicism.
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