Ichthyosis hystrix is a rare genodermatosis characterized by persisten
t spiny scales which cover a significant part of the skin surface. In
many cases it is associated with palmoplantar keratoderma, and occasio
nally deafness and neurological defects coexist. The case of a man wit
h ichthyosis hystrix is reported who developed skin malignancy and dys
plastic keratoses, a complication which has not previously been record
ed.