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

Abstract

Multiple basal cell carcinomas (BCCs) can result from a genetic disease, such as basal cell naevus 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. To evaluate QoL in patients with BCNS and HF-BCC and the impact of number of BCCs on QoL. Patients with BCNS were recruited through the Maastricht University Medical Centre (MUMC+), the online national platform for patients with BCNS and through the Dutch Society for Dermatology and Venereology. 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 predefined cutoff scores. Seventy-five patients with BCNS and 59 with HF-BCC were included. Patients with BCNS were often younger (median 48 vs. 74 years), 61% (n = 46/75) were women vs. 34% (n = 20/59) of patients with HF-BCC, and 48% (n = 36/75) had > 100 BCCs vs. 2% (n = 1/59) of patients with HF-BCC. A substantial proportion of patients with BCNS reported a severe impact on emotions and functioning [36% (n = 27/75) and 23% (n = 17/75), respectively] and mean (SD) 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% confidence interval 2.5-17.5). Among patients with BCNS, BCC burden was negatively associated with QoL, with significantly higher scores in patients with > 100 compared with < 30 BCCs [mean (SD) total score of 32.0 (19.6) vs. 15.8 (11.6)]. This trend was not observed in patients with HF-BCC. These findings highlight increased QoL impairment in patients with BCNS compared with others with multiple BCCs, especially with respect to emotional and functional wellbeing. 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 underscore that disease-related challenges in emotional and social domains should be considered during consultations to improve patient support.