Keywords: Dermatosis, otologic lesions

Abstract: The authors report a case of a patient with Pachyonichya Congenita Syndrome, a rare genodermatosis inherited as an autossomic dominant trait, who also had otologic lesions beyond the other classic signs and symptoms of the syndrome. Many kinds of treatment have already been proposed, but all failed to show satisfactory results. A new, cheap and easy-to-use treatment was developed in this study, using keratoblastics interpolated with humectant lotion for 90 days. The results after3 yean of follow-up are still thoroughly satisfactory.