title: Risk Factors for Basal Cell Carcinoma Among Patients With Basal Cell Nevus Syndrome: Development of a Basal Cell Nevus Syndrome Patient Registry.
abstract: OBJECTIVE
  Patients with basal cell nevus syndrome (BCNS) have a greater risk of developing numerous basal cell carcinomas (BCCs). Risk factors influencing the wide variation in tumor burden are poorly understood.
  
  
  OBJECTIVE
  To describe the burden of BCCs in patients with BCNS in the United States and identify potential risk factors for BCCs.
  
  
  METHODS
  Prospective clinical registry with data collected from September 2014 to March 2016. Participants were recruited from a mailing list of patients with BCNS at Children's Hospital Oakland Research Institute and Basal Cell Carcinoma Nevus Syndrome Life Support Network. Patients of all ages with a diagnosis of BCNS were eligible for enrollment. Participants completed a clinical questionnaire on their disease characteristics and risk factors.
  
  
  METHODS
  Number of BCCs in the past 2 years and over lifetime (disease burden), risk factors for BCCs.
  
  
  RESULTS
  A consecutive sample of the first 141 participants was included (34% [100 of 297] response rate from paper survey, 23% [41 of 179] from online survey; 85 [60%] female; mean age at start of study, 53 [range, 8-83] years; 131 [93%] white). In the previous 2 years, participants reported a mean of 25 BCCs (median, 11; range, 0-250). Over their lifetime, participants reported a mean of 257 BCCs (median, 160; range, 0-2200). Univariate analysis identified age (odds ratio [OR], 1.05; 95% CI, 1.03-1.07; P < .001), number of sunburns (OR, 1.05; 95% CI, 1.00-1.10; P = .047), and history of radiation exposure (OR, 2.26; 95% CI, 1.02-5.03; P = .046) as potential risk factors for lifetime BCC severity. On multivariate analysis, only age (OR, 1.04; 95% CI, 1.02-1.07; P < .001) and number of sunburns (OR, 1.06; 95% CI, 1.00-1.11; P = .04) were statistically significant. In our adjusted models, BCC burden increased by 4% per year of age and by 6% per number of sunburns.
  
  
  CONCLUSIONS
  Patients with BCNS have a high burden of BCCs. Age and number of sunburns were significantly associated with the severity of lifetime BCC. Further interventions to prevent and treat BCCs in patients with BCNS are needed.
authors.affiliation: Medical student, Stanford University School of Medicine, Stanford, California.
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Solis
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Gina P
authors.initials: GP
authors.lastname: Kwon
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Katherine J
authors.initials: KJ
authors.lastname: Ransohoff
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Shufeng
authors.initials: S
authors.lastname: Li
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Harvind S
authors.initials: HS
authors.lastname: Chahal
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Mina S
authors.initials: MS
authors.lastname: Ally
authors.affiliation: Department of Dermatology, MC Zuiderzee Hospital, Lelystad, the Netherlands.
authors.forename: Marieke A D
authors.initials: MAD
authors.lastname: Peters
authors.affiliation: Basal Cell Carcinoma Nevus Syndrome Life Support Network, Burton, Ohio.
authors.forename: Kristi
authors.initials: K
authors.lastname: Schmitt-Burr
authors.affiliation: Children's Hospital Oakland Research Institute, Oakland, California.
authors.forename: Joselyn
authors.initials: J
authors.lastname: Lindgren
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Irene
authors.initials: I
authors.lastname: Bailey-Healy
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Joyce M
authors.initials: JM
authors.lastname: Teng
authors.affiliation: Children's Hospital Oakland Research Institute, Oakland, California.
authors.forename: Ervin H
authors.initials: EH
authors.lastname: Epstein
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Jean Y
authors.initials: JY
authors.lastname: Tang