Squamous cell carcinoma arising from Keratitis-ichthyosis-deafness syndrome.

Inherited ichthyoses are rare genodermatoses caused by mutations in the genes involved in epidermal develop-ment. Keratitis–ichthyosis–deafness (KID) syndrome is a debilitating ectodermal dysplasia that predisposes patients to develop squamous cell carcinomas (SCC) in addition to leading to profound sensory deafness and erythrokeratoderma already present at birth (1–5). Re-cent reports have provided evidence that KID syndrome is caused by a mutation of connexin 26 (Cx26) (2–5), a gap junction protein, encoded by GJB2, with the ma-jority of patients harbouring the D50N mutation (2–8). Interestingly, a previous review suggested that the mis-sense D50N mutation in patients with KID syndrome is strongly connected with the development of skin SCC (1), although not every case develops malignancies (6, 7). We describe here a case of large areas of SCC arising from KID syndrome after severe infection. Our report also suggests that severe bacterial infection might be one of the reasons for the establishment of this aggres-sive skin cancer. CaSe RepORt