Clouston syndrome: an ultrastructural study

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.

[1]  L. Barness.  Recognizable Patterns of Human Malformations , 1977 .

[2]  A. Norins,et al.  Pachyonychia congenita and steatocystoma multiplex , 1977, Clinical genetics.

[3]  C. Scriver,et al.  Properties of hair keratin in an autosomal dominant form of ectodermal dysplasia. , 1972, American journal of human genetics.

[4]  S. Comaish,et al.  PROGRESSIVE CHANGES IN CUTICULAR PATTERN ALONG THE SHAFTS OF HUMAN HAIR AS SEEN BY SCANNING ELECTRON MICROSCOPY * , 1972, The British journal of dermatology.

[5]  Gol'dberg Mb,et al.  The characterization of hereditary abnormalities of keratin: Clouston's ectodermal dysplasia. , 1971 .

[6]  C. Scriver,et al.  The characterization of hereditary abnormalities of keratin: Clouston's ectodermal dysplasia. , 1971, Birth defects original article series.

[7]  S. Comaish,et al.  Scanning electron microscopy of normal and abnormal hair shafts. , 1970, Archives of dermatology.

[8]  M. Williams,et al.  Hydrotic ectodermal dysplasia--Clouston's family revisited. , 1967, Canadian Medical Association journal.

[9]  G. H. Stevenson,et al.  Family with Inherited Ectodermal Dystrophy. , 1945, Canadian Medical Association journal.

[10]  Clouston Hr.  THE MAJOR FORMS OF HEREDITARY ECTODERMAL DYSPLASIA : (With an Autopsy and Biopsies on the Anhydrotic Type). , 1939 .

[11]  H. R. Clouston.  THE MAJOR FORMS OF HEREDITARY ECTODERMAL DYSPLASIA : (With an Autopsy and Biopsies on the Anhydrotic Type). , 1939, Canadian Medical Association journal.

[12]  H. Joachim.  HEREDITARY DYSTROPHY OF THE HAIR AND NAILS IN SIX GENERATIONS , 1936 .

[13]  Clouston Hr.  A Hereditary Ectodermal Dystrophy. , 1929 .

[14]  H. B. Thompson.  HEREDITARY DYSTROPHY OF THE NAILS , 1928 .

[15]  A. Jacobsen.  HEREDITARY DYSTROPHY OF THE HAIR AND NAILS , 1928 .

[16]  N. Tobias.  HEREDITARY FAMILIAL DYSTROPHY OF THE NAILS , 1925 .

[17]  F. A. Murray.  CONGENITAL ANOMALIES OF THE NAILS. FOUR CASES OF HEREDITARY HYPERTROPHY OF THE NAIL‐BED ASSOCIATED WITH A HISTORY OF ERUPTED TEETH AT BIRTH , 1921 .

[18]  A. Barrett.  HEREDITARY OCCURRENCE OF HYPOTHYROIDISM WITH DYSTROPHIES OF THE NAILS AND HAIR , 1919 .

[19]  J. Eisenstaedt.  THREE CASES OF FAMILY DYSTROPHY OF THE HAIR AND NAILS , 1913 .