Keratosis follicularis (Darier-White disease), with an unusual palmoplantar keratoderma

A 38-year-old woman presented with widespread, hyperkeratotic papules and plaques that had been present since childhood. Her mother, brother, and son have similar lesions. A diagnosis of keratosis follicularis was made, which has been treated with isotretinoin. Palmoplantar lesions of keratosis follicularis have been described as discrete, punctate keratoses, hyperkeratotic papules, small pits, or keratin-filled depressions. This patient has an unusual palmoplantar keratoderma in association with keratosis follicularis. The lesions are elevated, discrete, filiform, hyperkeratotic spires, which coalesce into large, hyperkeratotic plaques on the palms and soles.