Factors affecting quality of life in epidermolysis bullosa

Abstract

Epidermolysis bullosa is a rare group of inherited disorders that manifests as blistering or erosion of the skin in response to little or no apparent trauma. Patients suffer from long-term physical, social and economic consequences. Despite the fact that quality of life assessment has rapidly become a significant outcome variable in dermatology research, only a few studies regarding epidermolysis bullosa patients are available. This paper reviews studies evaluating the effect of epidermolysis bullosa on quality of life in patients and caregivers in order to assess which factors can best describe their health-related quality of life. Currently, considerable deficits prevail in both the diagnosis and treatment of rare skin diseases. Moreover, the psychological and social morbidities very easily evade the eyes of clinicians. Comparison of the impact of epidermolysis bullosa with that of other conditions is clearly valuable for making public policy decisions. This review indicates the need for more rigorous exploration of the impact of such diseases to further guide treatment and to monitor quality of care.

Keywords::

• disability
• epidermolysis bullosa
• health outcome
• quality of life
• rare disease

Financial & competing interests disclosure

The study was financially supported in part by the ‘Progetto Ricerca Corrente 2009’ of the Italian Ministry of Health and in part by the Grant for Rare Disease (526D/4.2) of Istituto Superiore di Sanità, Rome. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.