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Keywords:

  • Clouston syndrome;
  • Hypohideotic Ectodermac Dysplasia

A previously undescribed French-Canadian family affected with Clouston Syndrome (Hypo-hidrotic 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.