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Congenital Ichthyosiform Erythroderma (CIE), also known as Nonbullous congenital ichthyosiform erythroderma〔Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.〕 is a rare type the ichthyosis family of skin diseases which occurs in 1 in 200,000 to 300,000 births. ==Symptoms== CIE has symptoms very similar to Lamellar ichthyosis (LI) but milder and is considered by many scientists to be a variant of that disease, so both diseases are grouped under the title autosomal recessive congenital ichthyosis (ARCI).〔(''Lamellar ichthyosis/CIE - New concepts, new mutations.'' ), Recent Ichthyosis Research, F.I.R.S.T.〕 The baby is often born in a collodion membrane, a shiny, wax outer layer on the skin and usually with ectropion, having the lower eyelid turned outwards. When the membrane is shed the skin is red with a generalized white scale. Palms, soles and areas on the joints are often affected with hyperkeratosis, a thickening of the layer of dead skin cells on the surface of the skin. In classical CIE (unlike LI) there is little eclabion (eversion of the lips), ectropion and alopecia (hair loss). Many people with ACRI don't fit neatly into the definition of LI or CIE but have characteristics of both diseases. The definitions of CIE and LI describe the extremes of the range of ACRI. 〔(''Congenital Ichthyosiform Erythroderma'' ), Types of Ichthyosis, F.I.R.S.T〕 〔(''Congenital Ichthyosis'' ), Rare Disease Registry, University of Padua, Italy〕 抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)』 ■ウィキペディアで「Congenital ichthyosiform erythroderma」の詳細全文を読む スポンサード リンク
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