title: Malignant skin tumours in patients with inherited ichthyosis.
abstract: Inherited ichthyoses are rare genodermatoses caused by mutations in the genes involved in epidermal development. Although there have been case reports on patients with ichthyosis who developed skin malignancies, it is still unknown whether or not patients with ichthyosis have an increased risk of skin malignancies. Here, we review case series of skin malignancies in patients with ichthyosis and show biological findings which might lead to cancer susceptibility. A survey of the literature revealed 28 cases of inherited ichthyoses with skin malignancy, including 12 cases of keratitis-ichthyosis-deafness (KID) syndrome, seven of autosomal recessive congenital ichthyosis, three of Netherton syndrome and six of miscellaneous ichthyosis. Twenty-four of the 28 cases developed single or multiple squamous cell carcinomas (SCCs). The age at diagnosis of the first skin malignancy ranged from 15 to 54 years. As patients with these particular subtypes of ichthyosis seem to be prone to skin malignancies, including SCC, at an unusually young age, routine cancer surveillance of these patients is strongly recommended.
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, North 15 West 7, Sapporo 060-8638, Japan. natsuga@med.hokudai.ac.jp
authors.forename: K
authors.initials: K
authors.lastname: Natsuga
authors.forename: M
authors.initials: M
authors.lastname: Akiyama
authors.forename: H
authors.initials: H
authors.lastname: Shimizu
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002294:Carcinoma, Squamous Cell / Q000235:genetics*; D006801:Humans; D007057:Ichthyosis / Q000235:genetics*; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D012307:Risk Factors; D012878:Skin Neoplasms / Q000235:genetics*; D055815:Young Adult