Health-related quality of life in patients with primary cutaneous amyloidosis

Abstract

Background Primary cutaneous amyloidosis (PCA) is a relatively rare and itchy skin disorder characterized by amyloid deposits in the superficial dermis. The cosmetic disfigurement and severe pruritus dramatically affects the patient's quality of life. In spite of the prevalence of the disease in China, the quality of life (QoL) impact of the PCA has not been well defined and is the focus of this study. Objective To examine the HRQoL of patients with PCA and to evaluate the association between HRQoL scores, disease, and socio-demographic determinants. Methods A total of 104 PCA patients and 101 healthy participants completed the questionnaires. HRQoL was measured using dermatology life quality index (DLQI) and SF-36. The socio demographic and clinical data such as age, sex, duration of disease and distribution of lesion pattern were analyzed mainly by hierarchical multiple regression analyses. Results Patients with PCA experienced significantly impaired health-related quality of life. The mean DLQI score was 9.05. Younger age, female gender, more pruritus and distribution pattern were independent predictor correlates of the high DLQI scores. The PCA group showed significantly decreasing average scores in several aspects of psychological symptoms, including SF, RE and MH. Conclusions PCA disease has a negative impact on the HRQoL of patients, and the HRQoL is associated with various disease characteristics. In conjunction with medical interventions, psychological functioning component of QoL and localization on the uncover area. Patients of PCA with lesion in uncover area had worse functional QoL. The association between impaired QoL and anatomical sites has already been demonstrated [4]. These findings suggest that anatomical locations should be incorporated into a severity scoring system as weight-assigned variables. As shown in our studies, it would be expected that localization of dermatological conditions on visible parts of the body, such as the face and hands, should impair QoL more than localization on hidden parts.Although PCA patients with a longer duration may have lower Qol, in our study, they did not impact the DLQI and SF-36. In the multifactorial model, duration of PCA was also not an independent predictor of either the DLQI or SF-36 score.The current study has some limitations. First, all subjects were managed by dermatologists in one city. Enrolled participants were from primary dermatology clinics and are therefore not representative of the general PCA population; this potentially could have influenced the DLQI score. Second, the study was cross-sectional and did not discriminate between those who had initiated treatment and those who had not. To fully explore the relationship between disease activity and QoL, future studies on the changes in QoL as disease activity changes are warranted. In addition, the study is limited by the small study population. A larger patient population needs to be studied to further assess QoL in patients with PCA, specifically to better compare PCA with other diseases.