Acrokeratoelastoidosis

Acrokeratoelastoidosis is a rare palmoplantar keratoderma. We report the case of a 32-year old man with multiple keratotic papules and plaques on the peripheral margins of his hands and feet. Histopathologic evaluation showed compact orthokeratosis; Verhoeff van Gieson stain showed thin and fragmented elastic fibers. The differential diagnosis includes focal acral hyperkeratosis, which has a similar presentation but with normal elastic fibers. Treatment of acrokeratoelastoidosis is often disappointing, with minimal response to topical glucocorticoids, keratolytics, retinoids, cryotherapy, and lasers.