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July 2006

Acrokeratoelastoidosis With Nail Dystrophy: A Coincidence or a New Entity?

Arch Dermatol. 2006;142(7):927-947. doi:10.1001/archderm.142.7.939-b

Acrokeratoelastoidosis (AKE) was first described in 1954.1 This dominant disorder is characterized by the presence of small, skin-colored to yellowish, round to polygonal papules on the thenar and hypothenar eminences of the palms and on the plantar surfaces of the feet. Histologic examination typically shows broadening of the granular layer, with circumscribed hyperkeratosis in a cup-shaped depression of the epidermis. Fragmentation of elastic fibers is also found.2 Histologic studies are needed to differentiate AKE from similar disorders such as focal acral hyperkeratosis, acrokeratosis verruciformis of Hopf, and punctate palmoplantar keratoderma.3,4 To our knowledge, no other skin abnormalities have been described in conjunction with AKE to date,4 but we recently saw a patient with AKE that was accompanied by pitted keratolysis and nail dystrophy.