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Pilosebaceous Dysplasia in the Oral-Facial-Digital Syndrome

Pilosebaceous Dysplasia in the Oral-Facial-Digital Syndrome Abstract The clinical features of eight female patients with the oral-facial-digital (OFD) syndrome, including a description of the skin and its histological and pharmacological characteristics are described. The OFD syndrome is characterized in part by a cutaneous dyplasia which includes a marked diminution in sebaceous glands, some decrease in hair follicles, and early keratinous cyst formation, which may represent maldevelopment of the pilosebaceous unit. References 1. Papillon-Léage Mme, Psaume J: Une ma[ill]f[ill]rmation hereditaire de la muqueuse buccale, brides, et freins anormaux: Generalites . Rev Stomatol 55: 209-227, 1954. 2. Ruess AL, Pruzansky S, Lis EF, et al: The oral-facial-digital syndrome: A multiple congenital condition of females, with associated chromosomal abnormalities . Pediatrics 29:985-995, 1962. 3. Gorlin RJ, Psaume J: Orodigitofacial dysostosis: A new syndrome: A study of 22 cases . J Pediat 61:520-530, 1962.Crossref 4. Gorlin RJ, Pindborg JJ: Syndromes of the Head and Neck . New York, McGraw-Hill Book Co, 1964, pp 438-446. 5. Fuhrmann W, Stahl A, Schroeder TM: Das Oro-facio-digitale Syndrom . Humangenetik 2:133-164, 1966. 6. Wahrman J, Berant M, Jacobs J, et al: The oral-facial-digital syndrome: A male-lethal condition in a boy with 47/XXY chromosomes . Pediatrics 37: 812-821, 1966. 7. Doege TC, Thuline HC, Priest JH, et al: Studies of a family with the oral-facial-digital syndrome . New Eng J Med 271:1073-1080, 1964.Crossref 8. Mohr OL: A hereditary sub-lethal syndrome in man . Skrifter Norske Videnske Acad 14:3-18, 1941. 9. Rimoin DL, Edgerton MT: Genetic and clinical heterogeneity in the oral-facial-digital syndromes . J Pediat 71:94-102, 1967.Crossref 10. Papillon-Léage Mme, Psaume J: Dysmorphie des frein buccaux . Actualites Odontostomat 25:7-26, 1954. 11. Lauterstein AM, Pruzansky S: Tooth anomalies in the oral-facial-digital syndrome . Teratology 2:137-145, 1969.Crossref 12. Patau K, Therman E, Inhorn S, et al: Partial-trisomy syndromes: II. An insertion as cause of the OFD syndrome in mother and daughter . Chromosoma 12:573-584, 1961.Crossref 13. Schwartz E, Fish A: Roentgenographic features of a new congenital dysplasia . Amer J Roentgen 84:511-517, 1960. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Pilosebaceous Dysplasia in the Oral-Facial-Digital Syndrome

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Publisher
American Medical Association
Copyright
Copyright © 1970 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1970.04000120016003
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Abstract

Abstract The clinical features of eight female patients with the oral-facial-digital (OFD) syndrome, including a description of the skin and its histological and pharmacological characteristics are described. The OFD syndrome is characterized in part by a cutaneous dyplasia which includes a marked diminution in sebaceous glands, some decrease in hair follicles, and early keratinous cyst formation, which may represent maldevelopment of the pilosebaceous unit. References 1. Papillon-Léage Mme, Psaume J: Une ma[ill]f[ill]rmation hereditaire de la muqueuse buccale, brides, et freins anormaux: Generalites . Rev Stomatol 55: 209-227, 1954. 2. Ruess AL, Pruzansky S, Lis EF, et al: The oral-facial-digital syndrome: A multiple congenital condition of females, with associated chromosomal abnormalities . Pediatrics 29:985-995, 1962. 3. Gorlin RJ, Psaume J: Orodigitofacial dysostosis: A new syndrome: A study of 22 cases . J Pediat 61:520-530, 1962.Crossref 4. Gorlin RJ, Pindborg JJ: Syndromes of the Head and Neck . New York, McGraw-Hill Book Co, 1964, pp 438-446. 5. Fuhrmann W, Stahl A, Schroeder TM: Das Oro-facio-digitale Syndrom . Humangenetik 2:133-164, 1966. 6. Wahrman J, Berant M, Jacobs J, et al: The oral-facial-digital syndrome: A male-lethal condition in a boy with 47/XXY chromosomes . Pediatrics 37: 812-821, 1966. 7. Doege TC, Thuline HC, Priest JH, et al: Studies of a family with the oral-facial-digital syndrome . New Eng J Med 271:1073-1080, 1964.Crossref 8. Mohr OL: A hereditary sub-lethal syndrome in man . Skrifter Norske Videnske Acad 14:3-18, 1941. 9. Rimoin DL, Edgerton MT: Genetic and clinical heterogeneity in the oral-facial-digital syndromes . J Pediat 71:94-102, 1967.Crossref 10. Papillon-Léage Mme, Psaume J: Dysmorphie des frein buccaux . Actualites Odontostomat 25:7-26, 1954. 11. Lauterstein AM, Pruzansky S: Tooth anomalies in the oral-facial-digital syndrome . Teratology 2:137-145, 1969.Crossref 12. Patau K, Therman E, Inhorn S, et al: Partial-trisomy syndromes: II. An insertion as cause of the OFD syndrome in mother and daughter . Chromosoma 12:573-584, 1961.Crossref 13. Schwartz E, Fish A: Roentgenographic features of a new congenital dysplasia . Amer J Roentgen 84:511-517, 1960.

Journal

Archives of Dermatology – American Medical Association

Published: Dec 1, 1970

References

  • Une ma[ill]f[ill]rmation hereditaire de la muqueuse buccale, brides, et freins anormaux: Generalites
    Papillon-Léage Mme, Psaume J
  • The oral-facial-digital syndrome: A multiple congenital condition of females, with associated chromosomal abnormalities
    Ruess AL, Pruzansky S, Lis EF, et al
  • Orodigitofacial dysostosis: A new syndrome: A study of 22 cases
    Gorlin RJ, Psaume J
  • Syndromes of the Head and Neck
    Gorlin RJ, Pindborg JJ
  • Das Oro-facio-digitale Syndrom
    Fuhrmann W, Stahl A, Schroeder TM
  • The oral-facial-digital syndrome: A male-lethal condition in a boy with 47/XXY chromosomes
    Wahrman J, Berant M, Jacobs J, et al
  • Studies of a family with the oral-facial-digital syndrome
    Doege TC, Thuline HC, Priest JH, et al
  • A hereditary sub-lethal syndrome in man
    Mohr OL
  • Genetic and clinical heterogeneity in the oral-facial-digital syndromes
    Rimoin DL, Edgerton MT
  • Dysmorphie des frein buccaux
    Papillon-Léage Mme, Psaume J
  • Tooth anomalies in the oral-facial-digital syndrome
    Lauterstein AM, Pruzansky S
  • Partial-trisomy syndromes: II. An insertion as cause of the OFD syndrome in mother and daughter
    Patau K, Therman E, Inhorn S, et al
  • Roentgenographic features of a new congenital dysplasia
    Schwartz E, Fish A