Juliane Schierbeck, Tine Vestergaard, Anette Bygum
DOI: 10.2340/00015555-3123

Skin cancer has become the most common type of cancer worldwide as a result of environmental exposure and medical treatments. A small group of patients are genetically predisposed to skin cancer and this article is intended as a diagnostic tool when encountering patients with multiple skin cancer lesions. The disorders are described with clinical characteristics, genetics and management. The most common syndromes associated with basal cell carcinoma are: Gorlin–Goltz syndrome, Rombo syndrome, and Bazex-Dupré-Christol syndrome. Multiple squamous cell carcinomas can be related to: xeroderma pigmentosum, Ferguson-Smith, Muir-Torre syndrome, Mibelli-type porokeratosis, keratitis-ichthyosis-deafness syndrome, Rothmund-Thomson syndrome, Bloom syndrome, and epidermodysplasia verruciformis. Malignant melanoma can be inherited, as in familial atypical multiple mole melanoma syndrome.

This article reviews hereditary skin syndromes that cause an increased risk of skin cancer development. It is important for physicians treating skin cancer to be aware of hereditary causes, especially when examining patients with multiple cancerous lesions with no obvious explanation. This article describes clinical features, genetic descriptions and management suggestions for hereditary syndromes associated with skin cancer, and includes clinical images from our practice.