Ectodermal Dysplasia Syndrome in Siblings with True Keloids, Stenosis of the Esophagus after Operations for Congenital Achalasia and Renovascular Hypertension due to Stenosis of Renal Artery

Naoya SHIMOHASHI; Masayuki FURUKAWA; Hiroya YAMAGUCHI; Toshihiko HASHIMOTO; Fumio UMEDA; Hajime NAWATA

doi:10.2169/internalmedicine.34.406

Naoya SHIMOHASHI, Masayuki FURUKAWA, Hiroya YAMAGUCHI, Toshihiko HASHIMOTO, Fumio UMEDA, Hajime NAWATA

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Keywords: hydrotic ectodermal dysplasia syndrome, autosomal recessive inheritance, EGF

JOURNAL FREE ACCESS

1995 Volume 34 Issue 5 Pages 406-409

DOI https://doi.org/10.2169/internalmedicine.34.406

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Abstract

Ectodermal dysplasia syndrome (EDS) is a rare hereditary disease, with symptoms brought about by dysplasia of ectodermal tissue (such as skin, teeth, nails, and hair). This report details the cases of two siblings (41 and 43 year old sisters) with autosomal recessive and hydrotic EDS complicated by esophageal achalasia, postoperative stenosis of esophagus, true keloids, renovascular hypertension, incomplete malrotation of the bowel, and demyelination of the brain.
(Internal Medicine 34: 406-409, 1995)

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