Abstract: A 61-year-old female presented with palmoplantar keratoderma, constricting bands on several fingers and joint contracture for 60 years. Clinical examination showed marked palmoplantar keratoderma, fibrous constricting bands on several fingers, brown polygonal scales on limbs. The patient had neither deafness nor dysgnosia. Her parents were cousins, and her sister had similar symptoms.

Key words: mutilanting palmoplantar keratoderma, ichthyosis