HANDICAP IN DARIERS-DISEASE AND HAILEY-HAILEY DISEASE

One hundred and thirty-seven patients with Darier's disease and 66 pat ients with Hailey-Hailey disease completed the Dermatology Life Qualit y Index (DLQI). The average DLQI score for Darier's disease was 5.89 ( 19.6% of the maximum score), median = 4, quartiles: 1,9. The average D LQI score for Hailey-Hailey disease was 6.06 (20.2% of the maximum sco re), median = 5, quartiles: 2,9. The DLQI score did not correlate with the physician's assessment of clinical severity in both diseases, thu s emphasizing the importance of remembering the potential for handicap even in patients with disease that seems to be mild. The domains of t he questionnaire with the highest mean scores in both diseases were fo r questions relating to symptoms and feelings. Domains relating to per sonal relationships and work obtained low mean scores even in severe d isease. It is encouraging that most patients with Darier's disease and Hailey-Hailey disease maintain good relationships at work and home an d seem to be able to adapt to their skin disease.