The overall objective of this guideline is to provide up-to-date, evidence-based recommendations for the diagnosis and surveillance of all symptoms in children and adults with either basal cell naevus syndrome (BCNS), a clinical suspicion of BCNS, or a parent with BCNS. In the last two groups, the guidelines should be followed until the diagnosis of BCNS can be rejected with certainty. The guideline aims to:• Update and expand on the previous guidelines by an appraisal of all relevant literature from January 2011 up to January 2021• Address important, practical, clinical questions relating to the primary guideline objective• Provide guideline recommendations • Discuss potential developments and future directions The guideline is presented as a detailed review with highlighted recommendations for practical use in the clinic by dermatologists and other healthcare professionals, including general practitioners, clinical geneticists, paediatricians, ophthalmologists, craniomaxillofacial surgeons, neurologists, cardiologists and psychologists.
ExclusionsThe guideline does not cover therapeutic recommendations for (nondermatological) symptoms, as the guideline mainly focuses on screening and follow-up of symptoms. Therapeutic recommendations for basal cell carcinomas (BCCs) in general have been published in international BCC guidelines. 1,2
Stakeholder involvement and peer reviewThe guideline was developed at the Maastricht University Medical Centre (MUMC+), the Dutch BCNS expert centre accredited