Management of Cutaneous Manifestations of Genetic Epidermolysis Bullosa: A Multiple Case Series.

BACKGROUND

Epidermolysis bullosa (EB) is a rare disease characterized by blistering and erosion of the skin and mucus membranes in response to minor external forces. Research focusing on daily skin care in EB patients is sparse. Two international clinical practice guidelines (CPGs) have been published in English, but they have not yet been translated into other languages such as Japanese. Therefore, their recommendations have not been adapted to multiple geographic regions in the world. This multiple case series describes our approach to the skin care of 3 Japanese patients with EB.

RESULTS

All 3 patients were diagnosed with genetic EB. A 13-year-old male patient had dominant dystrophic EB and suffered skin breakdown covering nearly 1% to 6% of his total body surface area (TBSA) during a 21-week data collection period. A 3-year-old male patient had EB simplex; he suffered skin breakdown covering 2% to 40% of his TBSA during a 36-week data collection period. The third case was a 5-year-old male patient with recessive dystrophic EB who experienced skin breakdown covering 2% to 40% of his TBSA during a 35-week data collection period. Blisters were punctured daily and treated with a soft silicone dressing. Daily application of moisturizers was undertaken to prevent the skin from drying out and itching.

CONCLUSION

Our experience suggests that application of published CPGs promoted wound healing. Nevertheless, given the nature of the disease, a complete resolution to an individual's vulnerability to skin lesions even with relatively minor trauma remains elusive. Additional research is needed to explore interventions for skin and ulcer care, along with symptom management, including pain and pruritus.