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

Abstract

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.

Impaired Dermatoscopic Visualization in a Patient With Ichthyosis Vulgaris Undergoing Complete Skin Examination

“…Similarly, filaggrin mutations are associated with greater risks for developing atopic dermatitis, a condition that is associated with higher risks for nonmelanoma skin cancer. Finally, some have speculated that the chronic inflammation associated with the impaired skin barrier in ichthyosis could contribute to carcinogenesis (Wakumoto et al, 2020). Despite these findings, the true relationship between ichthyosis and skin cancer risk remains unclear (Natsuga et al, 2011; Skaaby et al, 2014); however, given the potential association, some have suggested that patients with IV undergo lifelong skin cancer surveillance (Tas et al, 2017).…”