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Back to Dermatology Diseases
Epidermolytic hyperkeratosis
Epidermolytic hyperkeratosis, also known as bullous congenital
ichthyosiform erythroderma or simply bullous congenital ichthyosiform
erythroderma is a rare skin disease in the ichthyosis family affecting
around 1 in 250,000 people.
At birth, affected babies may have little or no top layer of skin, and
are therefore at high risk from infection. Subsequently, scaling
caused by hyperkeratosis is observed. There is a fairly large
variation in the degree and extent of the scaling. In particular, some
patients have scaling on the palms and soles of the feet whereas
others do not. Usually scaling is seen on the rest of the body, often
concentrated around the joints.
Additionally, patients periodically develop blisters (hence the
bullous part of the name). Typically these will be more common in
children than in adults.
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This is a dominant genetic condition.
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