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KMID : 0363219750130020145
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Korean Journal of Dermatology
1975 Volume.13 No. 2 p.145 ~ p.150
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Congenital Ichthyosiform Erythroderma
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高昌朝/Koh, Chang Jo
趙鼎九/禹泰河/Cho, Chung Koo/Woo, Tae Ha
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Abstract
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Congenital ichthyosiform erythroderma is most severe type of ichthyosis.
This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration.of the mid= and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover time.
This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local treatments. The autopsy findings had exhibited fatty necrotic degenerative´ changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
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