ABSTRACT
Darier disease and Hailey-Hailey disease are rare autosomal dominant genodermatoses that negatively impact patient quality of life. In addition, they pose challenges to dermatologists who manage these diseases. There is currently no treatment that reliably induces remission for either disease, leaving patients dependent on symptom management. Oral and topical retinoids are the most commonly used therapies but have numerous side effects that often lead to discontinuation or inability to tolerate long-term treatment (Burge and Wilkinson in J Am Acad Dermatol 27:40-50, 1992). Due to the rarity of these diseases, there are no clinical trials investigating treatment options for the persistent flares patients experience. In light of this, dermatologists have tried various methods used in the management of other inflammatory disorders including photodynamic therapy (PDT). A systematic review was conducted to investigate this treatment option which yielded a total of 12 studies that had reported the use of photodynamic therapy (PDT) as a treatment option for Darier or Hailey-Hailey disease. Though results showed that PDT can induce disease remission for up to several months or years, there are many unanswered questions that need to be addressed before adopting PDT as a leading treatment option for those genodermatoses. In particular, cost, tolerability, and efficacy and safety in patients who are skin of color need to be further studied. Lastly, recommendations on treatment duration, number of sessions, photosensitizing agents, lasers, and continuation or discontinuation of topical and systemic medications need to be appraised before formal recommendations can be made.
Subject(s)
Darier Disease , Pemphigus, Benign Familial , Photochemotherapy , Photosensitizing Agents , Humans , Darier Disease/drug therapy , Darier Disease/psychology , Pemphigus, Benign Familial/drug therapy , Pemphigus, Benign Familial/psychology , Photochemotherapy/methods , Photochemotherapy/adverse effects , Photosensitizing Agents/administration & dosage , Photosensitizing Agents/adverse effects , Quality of Life , Treatment OutcomeABSTRACT
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.
Subject(s)
Pemphigus, Benign Familial/psychology , Quality of Life , Stress, Psychological , Adult , Aged , Female , Humans , Male , Middle Aged , Pemphigus, Benign Familial/pathology , Severity of Illness Index , Surveys and QuestionnairesABSTRACT
We report on a family with co-occurrence of affective disorder and Hailey-Hailey disease in two brothers and the mother. The putative chromosomal locus of Hailey-Hailey disease, which is a rare dominantly inherited dermatosis, may be a promising candidate region for genetic studies in affective disorder.