title: Health-related quality of life among Darier's disease patients.
abstract: BACKGROUND
  Darier's disease (DD) is an autosomal dominant skin disorder characterized by persistent eruption of hyperkeratotic papules. The effect of DD on quality of life (QOL) has been assessed in only one study, which found no correlation between the Dermatology Life Quality Index (DLQI) score and clinical severity of the disease. The correlation between health-related quality of life (HRQL) and other diseases and patient characteristics has not been studied.
  
  
  OBJECTIVE
  To examine the HRQL of patients with DD and to evaluate the association between HRQL scores and disease and patient characteristics.
  
  
  METHODS
  A total of 74 DD patients completed three QOL questionnaires: DLQI, EQ-5D, and one specially designed for the study. The data reported in this study were collected as part of a larger study on the clinical characteristics of DD; the socio-demographic and clinical data were used in the statistical analysis of the current study.
  
  
  RESULTS
  Mean DLQI was 5.41 ± 5.57 and the mean EQ-Visual Analogue Scale (VAS), was 70.84 ± 19.25. DLQI and EQ-VAS were significantly associated with skin area affected, disease severity, age at onset of DD and a seborrhoeic distribution pattern of DD. Stepwise linear regression showed skin area affected to be the most significant variable in the predication of DLQI (beta = 0.183; SE = 0.04; P < 0.001), and disease severity the most significant variable in the predication of EQ-VAS (beta = -9.15; SE = 3.21; P < 0.006).
  
  
  CONCLUSIONS
  Darier's disease has a negative impact on HRQL of patients and the HRQL is associated with various disease characteristics, mainly skin area affected and clinical severity.
authors: Department of Dermatology, Ha'emek Medical Center, Afula, Israel.
authors: R
authors: R
authors: Dodiuk-Gad
authors: E
authors: E
authors: Cohen-Barak
authors: M
authors: M
authors: Ziv
authors: A
authors: A
authors: Shani-Adir
authors: B
authors: B
authors: Amichai
authors: A
authors: A
authors: Zlotogorski
authors: S
authors: S
authors: Shalev
authors: D
authors: D
authors: Rozenman
mesh_terms: D000222:Adaptation, Physiological; D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D007644:Darier Disease / Q000175:diagnosis* / Q000523:psychology*; D005260:Female; D006801:Humans; D007557:Israel; D016014:Linear Models; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011788:Quality of Life; D012720:Severity of Illness Index; D018434:Sickness Impact Profile; D013315:Stress, Psychological; D011795:Surveys and Questionnaires; D013997:Time Factors; D055815:Young Adult