Monday, July 22, 2024

Epidermolysis Bullosa Responses

Derek Kozar

Below is the original post

Seems like I am always on here asking for advice on some weird disorders

(myotonia congenita a few weeks ago), and here I am back again.

Has anyone dealt with Epidermolysis Bullosa? Check it out here

Any friction on the skin causes blisters, and this poor 11 year old male

has blisters mainly on his lower extremity, with his right ankle being
the worst. The main problem here is that he has a 30 degree
plantarflexion contracture ( not sure why or how this developed),
subsequently he inverts the ankle with internal rotation. He has had a
contstant “blister” over his lateral malleolus for as long as Mom can
remember, and they are now trying skin grafting in this area. He needs

heel cord lengthening, but they will not do surgery for fear that his
skin will never heal!

His dermatologist has refered him, just looking for ideas. I definately

need to accomodate his PF contracture with a heel wedge on the shoe to
start. I guess my main question is, has anyone dealt with this, and if
so, what type of interface material have you used??? I would like to
stop the inversion, but any pressure here will surely cause skin
breakdown…I don’t know if anything can be done, but any ideas would be


Interesting problem. As a PT, I would suggest straightening out the
plantarflexion contracture first, NOT accommodating his gait with a heel

wedge…because any short-term stretching you do with an orthosis will
counteracted by long-term (i.e. all day) positioning with his shoe,
presumably while he is in school. While I haven’t dealt with his
skin condition, I have dealt with significant (>30 degrees) contracture
management. Because he is so young, his heel cords should stretch
quickly. The first thing that comes to mind is serial casting. I would
just below knee first, bivalving it for hourly skin checks, wound care,
One PT I worked with even used AFO/splinting material with a sort of
cut into it with adjustable tension to increase as the contracture
decreased. It was sort of like a custom-molded AFO Dynasplint. This
patient also had significant skin issues as he had fresh fasciotomy
This is preferred over pre-fabs because you can use thick
cast padding, webril, or lamskin to pad, also you can customize the fit
better skin contour. If the below-knee splint doesn’t do the trick, you
pull the ankle into max dorsiflexion, cast up to the knee, then extend
knee and cast above. I’ve done this, too. I would just make him
non-weight-bearing and have him use crutches. He’s 11. This shouldn’t
be a
problem for him. I would expect significant results in a week or two.
just need good cooperation from the family and school nurse.

Am J Phys Med Rehab 1988 Jun ; 67(3) :104-107
Successful prosthetic fitting of a patient with epidemolysis bullosa
dystrophica. Case Report
Jain SS , Delisa JA .

Consider Shear Guard by Becker. Most ulceration problems are more of a
result of friction versus direct pressure issues.

I am not sure if this will help, but I work with burn patients and we
Silicone Sheeting to cover areas that are highly sensitive. Just a

Derek, I have dealt with a young lady with this terrible disease for
18 years till last week when she died of pneumonia.
All orthotics were lined with any of the foam padded napped type
interfaces…these included cotton wools, ‘moleskin’ and many of the
proprietary linings.
None of these contacted the skin directly however, because the patient
always had her own dressings covered by crepe bandages. None of these
orthoses were therefore able to be close fitting and the wearing of all
them was subject to the various other difficulties that my patient had.

Serial casting not an option here….

Physio and Dr. now want to add heel wedge to accomodate the PF
contracture. Will start with this and go from there….

Thanks for your thoughts



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