A case of Buschke-Fischer-Brauer punstate keratodemia [Un caso di cheratodermia punctata di Buschke-Fisher-Brauer]

Buschke-Fisher-Brauer palmo plantar keratoderma is a rare autosomal dominant skin disease, currently classified among the circumscribed punctate forms. The underlying genetic mechanisms are not yet known. Onset is usually late with a peak incidence in the second decade of life; the disease is chronic and generally without subjective symptoms. Clinically it is characterized by small roundish yellow-brownish keratotic papules, often with a central crater-like depression, situated exclusively on the palms and soles. Various other diseases, such as ungual dystrophy, early greying of the hair, sebaceous hyperplasia, spastic paralysis, ankhylosing spondylitis and cancer, have been described in association with Buschke-Fisher-Brauer palmo plantar keratoderma. Ungual dystrophy is of interest because of its frequency and cancer because of its potential gravity. A case of Buschke-Fisher-Brauer palmo-plantar keratoderma in a 76-year old man is reported. The lesions had begun 45 years before. Six other members of his family had been affected over three generations. Four of these had died of concomitant tumours: 2 of colonrectal cancer and 2 of breast cancer.