Type I hereditary punctate keratoderma

Abstract

A 75-year-old man with a history of prostatic carcinoma and atypical fibroxanthoma reports a long-standing history of 1-2 mm depressed, hyperkeratotic papules on the palms. His mother suffered a similar condition. Histologically, the papules demonstrated hyperkeratosis, without columns of parakeratosis. A diagnosis of type I hereditary punctate keratoderma (Buschke-Fisher-Brauer disease) was made. This condition, which is classified as one of the three hereditary forms of punctate palmoplantar keratoderma, is an autosomal-dominant condition with variable penetrance. It is characterized clinically by multiple, tiny, punctate keratoses of the palms and soles. Affected individuals appear to be at increased risk of developing malignant conditions.