What are treatment options for dystrophic epidermolysis bullosa?

Updated: Jan 30, 2020
  • Author: Ginard I Henry, MD; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Answer

Answer

While no medical or surgical cure exists for this affliction, some modicum of benefit has been discovered with the vigorous use of topical steroid therapy. Surgical treatments are frustrating, and one of the larger series of these treatments has been developed at the Ormond Street Hospital for Children in London. In this facility, a highly specialized multidisciplinary approach is used in the perioperative setting to protect all of the epithelial tissues.

One significant clinical contribution from these large series has been the knowledge that within the epithelial cocoon, the digits remain surprisingly mobile, with a greater than expected active and passive range of motion. Skin grafts are used to close the intervening epithelial defects, and they actually heal quite normally. Generally, the donor site heals in an essentially typical fashion.

Another significant clinical observation has been the chronically colonized condition of the blister fluid with beta-hemolytic streptococci. Despite early excellent successes with surgical management, the final common pathway appears to be a recrudescence of the recurrent minor trauma and epithelial encasement. For this reason, treatment is considered only temporary.


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