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Bullous Congenital Ichthyosiform Erythroderma

Bullous congenital ichthyosiform erythroderma is a rare autosomal dominant condition where patients have generalized foul smelling and fissured hyperkeratotic skin at birth. It is characterized by erethroderma and severe hyperkeretosis, with distinctive, parallel ridges developing over joints. While erethema decreases after infancy, hyperkeratosis persists throughout life and recurrent blistering may occur in neonates, becoming accentuated with trauma but usually resolving by age 7-8.

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Abdiaziz Walhad
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72 views12 pages

Bullous Congenital Ichthyosiform Erythroderma

Bullous congenital ichthyosiform erythroderma is a rare autosomal dominant condition where patients have generalized foul smelling and fissured hyperkeratotic skin at birth. It is characterized by erethroderma and severe hyperkeretosis, with distinctive, parallel ridges developing over joints. While erethema decreases after infancy, hyperkeratosis persists throughout life and recurrent blistering may occur in neonates, becoming accentuated with trauma but usually resolving by age 7-8.

Uploaded by

Abdiaziz Walhad
Copyright
© Attribution Non-Commercial (BY-NC)
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPT, PDF, TXT or read online on Scribd
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Bullous congenital

ichthyosiform
erythroderma

(Epidermolytic
hyperkeratosis)
 It is a rare autosomal dominant
condition characterized by generalized
foul smelling and fissured
hyperkeratotic skin.

 Clinical manifestations are


characterized by the onset at birth of
generalized erethroderma and severe
hyperkeretosis.
The scales are small, hard,verrucous;
distinctive, parallel,hyperkeratotic
ridges develop over the joint flexures
,including the axillary, poplitial, and
antecubital fossas,and on the neck and
hips.
Erethema becomes less prominent after
infancy.However,the hyperkeratosis
persists throughout adult life.
Recurrent blistering may be widespread
in neonates and may cause diagnostic
confusion with other blistering
disorders.
Blistering becomes accentuated at sites
of trauma such as the knees ,elbows
and lower limbs but is not problematic
after age 7-8 yrs.
 The palms and
soles may be
thickened, but the
hair, nails, mucosa
and sweat glands
are normal.
 Secondary bacterial
infection is common

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