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Hidrotic Ectodermal Dysplasia with Ichthyosiform Erythroderma-like Skin Changes -An Autopsy Case-


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Title: Hidrotic Ectodermal Dysplasia with Ichthyosiform Erythroderma-like Skin Changes -An Autopsy Case-
Authors: Yokota, Minoru / Kawai, Kioko / Matsuda, Joshu / Kumagai, Kenji / Matsuo, Kenji / Tsuchiyama, Hideo
Authors (alternative): 横田, 実 / 河合, 紀生子 / 松田, 静宗 / 熊谷, 謙治 / 松尾, 健治 / 土山, 秀夫
Issue Date: 25-Oct-1984
Citation: Acta medica Nagasakiensia. 1984, 29(1-4), p.91-99
Abstract: We present an autopsy case of 30-day-old female infant who had hidrotic ectodermal dyplasia with ichthyosiform erythroderma-like skin changes and low value of migration test of neutrophils. At the time of birth she had the abscence of hairs, eyelashes, eyebrows, dystrophic nails, erythema and hyperkeratosis with scales on whole body. Autopsy revealed the presence of eccrine glands and hyperkeratosis of the epidermis, thymic involution and papillary proliferation of squamous epithelium in esophagus and vagina. Aspiration pneumonia is the immediate cause of her death. The co-existence of ectodermal dysplasia and ichthyosiform-skin changes in this case provide an opportunity to study the relationship between congenital dysplasia and ichthyosiform erythroderma in their development. It might also be possible to correlate that relationship on common ground of immunological insufficiency.
URI: http://hdl.handle.net/10069/15659
ISSN: 00016055
Type: Departmental Bulletin Paper
Text Version: publisher
Appears in Collections:Volume 29, No. 1-4

Citable URI : http://hdl.handle.net/10069/15659

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