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Elena Pope, MD, The Hospital for Sick Children, Toronto, ON Epidermolysis bullosa (EB) is a family of blistering disorders that can affect children and adults alike. The condition is inherited and causes blister formation with minor friction of the skin, causing painful, open wounds. Patients with this condition can be mildly affected (have only few blisters on the hands and feet) or severely affected with blistering involving the entire body with a poor quality of life and a decreased survival. In its most severe form the condition is fatal in infancy. So far, research in this field has been difficult as the number of patients is small with multiple complications and variable symptoms. However, the advances in understanding the molecular basis of EB are very promising and will yield more trials in the future. Further development will therefore require reliable, validated outcome measures to enable researchers to better interpret a particular intervention. The main aim of this study is the development and evaluation of an EB specific disease severity score that will facilitate future studies in this population. © 2009 |
