Facial photoprotection in xeroderma pigmentosum (XP) patients: Validation of a new self‐reported questionnaire of adherence

Abstract

Background/Purpose Adherence to photoprotection is the only way to prevent skin cancers and eye disease in xeroderma pigmentosum (XP). No validated self‐report questionnaire exists for assessing adherence to photoprotection practices in individuals with XP. We sought to validate a self‐reported measure of adherence to face photoprotection in this population. Methods Sixty six XP patients recruited from the patient list of the XP specialist service in London, UK, completed a questionnaire of adherence to specific photoprotection behaviours. We measured objective ultraviolet radiation (UVR) exposure to the face continuously for 21 days with a wristworn UVR electronic dosimeter combined with a daily photoprotection diary. Reliability and convergent validity of the questionnaire were tested in relation to overall UVR exposure, UVR dose to the face, daily photoprotection activities, other self‐reported photoprotection practices and clinical ratings of patient's protection. Results Internal consistency of the questionnaire was satisfactory. Questionnaire total scores were concordant with objective UVR exposure and UVR dose to the face. However, not all participants who reported good/excellent face photoprotection on the questionnaire recorded high levels of photoprotection in the daily diary. Correlations between the questionnaire and other practices and the clinical rating ranged from small to large in size. There was no correlation between the level of face photoprotection and self‐reported avoidance of going outside. Conclusions Our questionnaire was reliable and had good convergent validity with other indicators of photoprotection. This questionnaire could assist clinicians to detect low levels of adherence, and the methodology used to develop validated questionnaires for other photosensitive conditions.

Improving photoprotection in adults with xeroderma pigmentosum: personalisation and tailoring in the ‘XPAND’ intervention

“…Additional themes relevant to photoprotection and illness adjustment were the experience and management of stigma (see Anderson et al, 2017 ) and the importance of social support (see Walburn et al, 2019c ). The cross-sectional survey (see Walburn et al, 2019a ) involved completion of a purpose-designed adherence to face and body photoprotection measure, where a total score was calculated by considering the relative protection afforded by different protection combinations, plus a single-item to measure avoidance of going outside as a means of protection (see Canfield et al, 2019 ). Participants completed a series of validated self-report questionnaires to measure self-efficacy, automaticity, intention to protect, illness perceptions (consequences, timeline, personal control of XP, photoprotection control of XP, treatment control, identity, negative emotional representation, and perceived understanding), perceived effectiveness and necessity of protection, concerns about protection, and social support.…”

Lentigo maligna in a patient with xeroderma pigmentosum, variant type: A case report with dermoscopic findings and review of the literature

“…To the Editor, Xeroderma pigmentosum (XP) is a rare autosomal recessive genetic disorder affecting the DNA repair system, which is vital to mend the damage caused by ultraviolet (UV) radiation. 1,2 XP patients usually develop multiple skin tumors including basal cell carcinoma (BCC), squamous cell carcinoma (SCC), malignant melanoma (MM), and angiosarcoma. 3,4 However, the diagnosis of these tumors is often difficult due to severe actinic damage caused by the UV radiation.…”