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We report a case of X-linked recessive ichthyosis with basal cell carcinoma. An 86-year-old man was referred to our department for evaluation of a 1-year history of an ulcer on his left leg. He had suffered from dry skin with scales on his entire body since childhood. Histopathological examination of the lower leg revealed intradermal proliferation of a solid nest of basaloid cells showing unclear palisading in the periphery. Marked hyperkeratosis and hypergranulosis were also seen in the adjacent dermis. Loss of the steroid sulfatase gene was found on chromosome X. Based on these finding, we made a diagnosis of X-linked recessive ichthyosis with basal cell carcinoma. To our knowledge, there has been no report of X-linked recessive ichthyosis with cutaneous malignancy. We discuss the risk of skin cancer in relation to ichthyosis.

Is X-linked Recessive Ichthyosis a High Risk for Basal Cell Carcinoma?