Lamellar ichthyosis and non-bullous
ichthyosiform erythroderma
Understandably,
these rare conditions have often been confused in the past. Both may be
inherited as an autosomal recessive trait, and in both the skin changes at
birth are those of a collodion baby . Later the two conditions can be
distinguished by the finer scaling and more obvious redness of non-bullous
ichthyosiform erythroderma. Both last for life and are sufficiently disfiguring
for the long-term use of acitretin to be justifiable (Formulary 2). Lamellar
ichthyosis shows genetic heterogeneity: the most severe type is caused by
mutations in the gene for keratinocyte transglutaminase, an enzyme that
cross-links the cornified cell envelope, lying on chromosome 14q11.2.
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