What is the presentation and clinical progression of 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

Of greatest clinical significance is the involvement of children's hands. A continued unrelenting cycle of injury followed by scarring and contracture with creeping epithelialization occurs until the interdigital and web spaces are completely encased in an epidermal cocoon. A progressive atrophic scar results in the stepwise and eventual total loss of use of the digits of the hand.

While recognizing that variable clinical expression of this disorder exists, all individuals with dystrophic epidermolysis bullosa have some degree of functional loss. While the mucosal services of the hypopharynx and esophagus also are involved, they appear to be less significantly involved clinically than the exposed surfaces of the hands and fingers.


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