Severe impairment of quality of life in Hailey-Hailey disease

Abstract

Hailey-Hailey disease (a skin fragility disorder) runs a chronic course and may cause important disability. However, little has been formally investigated concerning the quality of life (QoL) of patients affected by this disorder. We studied the impact of Hailey-Hailey disease on the QoL of 22 consecutive patients seen at our dermatological clinic. Patients were examined by a dermatologist, and they were asked to complete two self-administered questionnaires: the Skindex-29, in order to determine their QoL, and the 12-item General Health Questionnaire, to evaluate psychological distress. Completed questionnaires were returned by 20 patients. Even in patients with few body sites involved, median overall QoL scores were much higher than those observed in other skin conditions in all three domains investigated by the Skindex-29 (i.e. symptoms, emotions and social functioning). This was true also for the levels of psychological distress. QoL impairment was substantial irrespective of the number of body sites involved. Our findings document a great impact of Hailey-Hailey disease on patients' QoL. Therefore, a more aggressive therapeutic approach may be warranted in all patients, including those with few lesions.