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Ichthyosis en confetti, also known as ichthyosis with confetti.
congenital reticular ichthyosiform erythroderma (CRIE) and
ichthyosis variegata, is a very rare form of congenital ichthyosis
in which healthy patches of normal skin co-exist within the
abnormal skin areas. It has been hypothesized that this is the
result of a combination of mitotic recombination and natural
selection within the skin. Choate et. al. have shown that mutations
in a gene called keratin-10 cause this disorder. They show that
mutant keratin 10 accumulates in the nucleolus, a sub-nuclear
structure, rather than within cellular intermedite filaments like
the wild-type protein. It has been speculated that the presence of
mutantkeratin 10 results in increased rates of mitotic
recombination, thus in effect leading the disease to effect its own
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