Health-related quality of life in patients with basal cell nevus syndrome and high-frequency basal cell carcinoma: a questionnaire study

BACKGROUND: Multiple basal cell carcinomas (BCCs) can result from a genetic disease, such as basal cell nevus syndrome (BCNS) or develop without a genetic predisposition, known as high-frequency BCC (HF-BCC). Multiple BCCs require frequent interventions, which may impact quality of life (QoL). Data on QoL in these patients and the influence of number of BCCs on QoL remain scarce. OBJECTIVES: To evaluate QoL in patients with BCNS and HF-BCC and the impact of number of BCCs on QoL. METHODS: Patients with BCNS were recruited through the Maastricht University Medical Centre (MUMC+), the online national BCNS patient platform and through the Dutch Society for Dermatology and Venereology (NVDV). Patients with HF-BCC (≥9 BCCs in 3 years or ≥6 in 10 years) were recruited from the MUMC+ and Catharina Hospital Eindhoven. QoL was measured with the Skindex-29 and severity of QoL impact was calculated based on pre-defined cut-off scores. RESULTS: Seventy-five patients with BCNS and 59 with HF-BCC were included. Patients with BCNS were often younger (median 48 versus 74 years), 61%(46/75) were female versus 34%(20/59) of patients with HF-BCC, and 48%(36/75) had > 100 BCCs versus 1.7%(1/59) of HF-BCC patients. A substantial proportion of patients with BCNS reported severe impact on emotions and functioning (36.0%(27/75) and 22.7%(17/75), respectively) and mean scores of 32.2±20.8 and 18.9±19.6 respectively. Adjusting for age and sex, the overall Skindex-29 score was higher in the BCNS group, with a between group difference of 10 (95% CI 2.5-17.5). Among patients with BCNS, BCC burden was negatively associated with QoL, with significantly higher scores in patients with >100 compared to <30 BCCs (mean total score of 32.0±19.6 versus 15.8±11.6). This trend was not observed in HF-BCC. CONCLUSIONS: These findings highlight an increased QoL impairment in patients with BCNS compared to others with multiple BCCs, especially with respect to emotional and functional well-being. This can be partly explained by the high BCC burden in this patient group. Our findings suggest that a high BCC burden is an indicator of psychosocial burden, and underscores that disease-related challenges in emotional and social domains should be considered during consultations to improve patient support.