Background: Epidermolysis bullosa (EB) is a group of
rare genetic disorders known by susceptibility of the skin
epidermis and mucosa that separate from underlying tissues
and cause increase in skin fragility, blistering, and scarring
in response to minor traumatic injuries. Many methods were
described in the literatures for soft tissue coverage after the
release of mitten hands which include cultured keratinocytes;
using epithelium raised from cocoon, to split skin grafts or
full-thickness skin grafts; and cellular allograft dermal matrix.
In this study, we described a simple and efficient noncoverage
technique, as it eliminates the donor site complications
when using STSG or FTSG, and also minimizes cost
management by avoid using cellular allograft dermal matrix,
specially in developing countries, where cost effectiveness
due to financial issues should be considered.
Aim of this Study: Is to describe and evaluate the noncoverage
technique based on the Birmingham Epidermolysis
Bullosa Severity Score Sheet (BEBSS) in patients suffering
from epidermolysis bullosa mitten hands.
Patients and Methods: We operated upon nine patients
suffering from epidermolysis bullosa mitten hands by using
the non-coverage technique to obtain full release of all fingers
and web spaces. Evaluation and scoring of preoperative and
postoperative hand scars were based on the 'Birmingham
Epidermolysis Bullosa Severity Score Sheet.
Results: Showed major improvement in the mean of postoperative
BEBSS with lower rate of recurrence and postoperative
complications. Hand function was remarkably improved
within 2 months which was demonstrated subjectively by the
capability of grasping and pinching objects such as cups,
spoons and pens using unilateral hand.
All patients and care givers were satisfied with the improvement
in function and the ability to perform more tasks
than preoperatively.
Conclusion: Managing soft tissue defects after release of
EB mitten hands can be left with no coverage and with
appropriate dressing for self-re-epithelialization can be ideal
method especially in developing countries where allografts
are relatively expensive and not available.