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 EB
Info World
The Children with EB
are called "Butterfly Children" because in spite of the extreme
fragility of their skin (like a butterfly wing), that blisters at the
slightest touch, they take on life and light it up with their bravery.
Th utterfly
Children™ is a registered trade mark of DEBRA UK
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Recessive Dystrophic -
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We
hope the pictures of these special children will touch your heart.
Scarring and milia
are the hallmarks of dystrophic EB of both the dominant and recessive
types. Recessive Dystrophic is particularly severe and mutilating. Large
bullae present at birth and heal slowly, with a thick crust and scars.
Fusion of the digits and mittenlike scars result encasing the hands and
feet. Blistering of the oral mucosa, larynx, and eye may cause great disability
as well. Nutrition is often a difficult problem, with poor intake and
increased protein loss through the skin complicated by anemia and chronic
constipation. Squamous Cell Carninoma (skin cancer) of the skin or mucosa
are seen in early adulthood and are a common cause of death. Most patients
with moderate to severe forms of Recessive Dystrophic EB rarely reach
their 30th birthday, and can succumb from reasons as varied as severe
anemia to heart failure. Please visit the EB memorial page to meet some of these patients that are no longer with us. The
fragility of the skin is also explained by the observation that anchoring
fibrils are decreased or absent in lesional and nonlesional skin in Recessive
Dystrophic Epidermolysis Bullosa.
Dominant Dystrophic
EB is generally milder than the recessive form. The Cockayne-Touraine
type has predominant involvement of extensor skin surfaces and bony prominences.
Presentation may be delayed until later infancy and childhood. Teeth are
normal and nails may be dystrophic. Healing areas may become thickened
and hypoplastic. The Pasini type also has extremity predominance, although
it may be generalized. Scarlike albopapuloid lesions are characteristic
in adolescence.
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