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Congenital ichtyosiform erythroderma, is a very rare type of ichtyosis in which the affected baby is usually born encased in a collodionlike membrane. Once the membrane has been shed, the skin remains more or less erythematous, covered with scales of various colors and sizes. Several phenotypes have been described depending on the presence of erythema, the size of scales, the presence of palmar or plantar lesions, the degree of ectropion (reverted eyelids), the affection of the scalp, the intensity of prurit and of intolerance to heat. Mutations in the gene coding for transglutaminase I have been mapped on chromosome 14. Another gene implied in the disease is located on chromosome 2. |