Risk factors for exacerbation of skin in patients with epidermolysis bullosa

Background: Epidermolysis Bullosa (EB) is one of rare genetic diseases that cause skin blistering and erosion repeated over patient’s lifetime with minor external force. Thus, EB is an orphan disease with less information on their daily skincare. Two international clinical practice guidelines have been published in recent years, but not yet to be translated. Also, it is difficult to apply the guidelines to patients because of difference in language and approved medical devices depending on one’s environment.

Aims: The aim of this study is to extract risk factors for exacerbation of skin condition of patients with EB to find out preventive measures.

Methods:  This qualitative - descriptive case study highlights to extract risk factors for exacerbation of skin condition. Patient’s symptom and their daily care methods were observed at scheduled outpatient clinic, and also, their skin conditions were recorded by photo images and descriptive note once in a week. Then, the methods of their future daily skincare using International Clinical Practice Guideline as a reference were discussed.   

Results:  Three cases were recruited. Patient A, 13-year-old male, who has EB simplex suffered skin break-down of 1~6% in Body Surface Area (BSA) for 5 months study period. Patient B, 3-year-old male, who has EB simplex suffered skin break-down of 2~40% in BSA for 9 months study period. Patient C, 5-year-old male, who has dystrophic EB suffered skin break-down of 2~40% in BSA for 9 months study period.  

Conclusion: This study highlights the importance of causative factors which lead to deteriorate of their skin. Itch and scratch made new blistering and erosion immediately after the wound had been once healed, in all patients.

This poster demonstrates with photos the visual appearance of the skin condition with EB.