A previously undescribed French-Canadian family affected with Clouston Syndrome (Hypohidrotic Ectodermal Dysplasia) is described. Ultrastructural study of the hair shows disorganization of the hair fibrils with loss of the cuticular cortex. The SEM findings are consistent with the model, suggesting a biochemical defect in the keratin of the integumentary system.
|Original language||English (US)|
|Number of pages||7|
|State||Published - Aug 1983|
- Clouston syndrome
- Hypohideotic Ectodermac Dysplasia
ASJC Scopus subject areas