Abstract
It is well known that patients with heritable cutaneous disease have an increased risk of developing tumors during their lifetime. This relationship has been proven for conditions such as xeroderma pigmentosum, Basal cell nevus syndrome, and Blooms syndrome. Multiple cutaneous tumors with Lamellar Ichthyosis had been reported in people with sun exposed skin. This article describes a case of genital squamous cell carcinoma in a patient with Lamellar Ichthyosis, which in our knowledge, described for the first time. During the long standing follow-up of patients with Lamellar Ichthyosis, careful attention should be paid to the occurrence of skin cancers in both, sun exposed and sun protected skin.
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