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Abstract

Background  Nevoid basal cell carcinoma syndrome (NBCCS) is a rare genetic disease which causes a variety of dermatological lesions, especially basal cell carcinomas (BCCs), often on the face, neck, and head.

Methods  Persons attending a national NBCCS support group meeting were asked to participate in survey-based assessments of quality of life and depressive symptoms. Inclusion criteria required a self-reported NBCCS diagnosis, voluntary agreement to participate, and age over 18 years. Exclusion criteria included cognitive impairment. Skin-related quality of life was assessed with Skindex-29, completed by 32 participants. Depressive symptomatology was determined with the Center for Epidemiological Studies Depression Scale (CES-D), completed by 18 participants. Sociodemographic, medical, and social variables were also analyzed.

Results  Median Skindex-29 scores for the emotions, symptoms, and functioning scales were 42.50, 32.14, and 28.13, respectively (means: 41.17, 37.05, and 29.30, respectively). These scores were slightly higher than those observed in patients with neurofibromatosis type 1, a similar genetic disease with skin symptoms. The CES-D scores (median = 15.50, mean = 17.50) suggested that 50% of participants had significant depressive symptomatology. Variables showing moderate associations with the scores included diet, number of affected family members, and treatment type. Interestingly, the number of BCCs had no effect.

Conclusions  Nevoid basal cell carcinoma syndrome impacts the quality of life of its subjects in a similar manner to other genodermatoses. Depressive symptoms are particularly prevalent. Several demographic, medical, and social characteristics affect these outcomes. Thus, the psychological impact of this disorder should be evaluated in the course of considering the care of persons with NBCCS.