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Epidermolysis bullosa simplex
Epidermolysis bullosa
simplex
, is a disorder resulting from mutations in the genes
encoding
keratin 5
or
keratin 14
.
Blister
formation
of
EBS
occurs at the
dermoepidermal junction
. Sometimes EBS is called
epidermolytic
.
Cause
Absence of keratin-5,14 since birth.
Diagnosis
Classification
Epidermolysis bullosa simplex may be divided into
multiple
types:
Type
Locus & Gene
OMIM
Epidermolysis bullosa simplex with migratory circinate
erythema
12q13
Epidermolysis bullosa simplex with mottled pigmentation.
Management
No cure for
EB
Treat
symptoms
Protect
skin, stop
blister
formation, promote healing
Prevent
complications
Necessary
treatment: use
oral
and
topical steroid
for healing and
prevent
complication
Maintain
cool
environment
,
avoid
overheating
and decreases friction