Somatic studies have revealed homozygous mutations of CYLD in these tumours, suggesting a ‘two-hit’ mechanism where an initial pathogenic germline mutation is followed by the acquisition of a second pathogenic somatic mutation, leading to the development of cutaneous adnexal tumours and in less frequently, basal cell adenomas of the salivary glands can transform into adenocarcinomas [3]. This evidence concerns the gene CYLD and neoplasm.