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Preauricular Skin Defects: A Consequence of a Persistent Ectodermal Groove

Preauricular Skin Defects: A Consequence of a Persistent Ectodermal Groove Abstract Background: The term aplasia cutis is used to describe congenital localized defects of the skin. This affliction is the end result of various in utero events. Aplasia cutis of the face, although rare, has been associated with numerous dysmorphic features and described under many clinical terms. Observations: We studied 10 patients with oval, atrophic patches distributed in a linear pattern on the preauricular region of the face. Most of the defects were bilateral, and all consistently fell in an oblique line extending from the preauricular region to the angle of the mouth. This line corresponds to the region of fusion between the maxillary and mandibular facial prominences during embryonic development. Conclusions: This type of facial aplasia cutis may be the result of incomplete fusion of the ectodermal groove between the maxillary and mandibular facial prominences. Although other types of facial skin defects may share a similar pathogenic mechanism, they are distinct in that they occur in different regions and may have other abnormal facial features.Arch Dermatol. 1997;133:1551-1554 References 1. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification . J Am Acad Dermatol. 1986;14:646-660.Crossref 2. Drolet BA, Prendiville J, Golden J, Enjolras O, Esterly N. Membranous aplasia cutis with hair collars: congenital absence of the skin or neuroectodermal skin defect? Arch Dermatol. 1995;131:1427-1431.Crossref 3. Gosain AK, Moore FO. Embryology of the head and neck . In: Aston SJ, Beasley RW, Thorne CH, eds. Grabb and Smith's Plastic Surgery . 5th ed. Boston, Mass: Little Brown & Co. In press. 4. Moore KL. The branchial apparatus and the head and neck . In: Moore KL, ed. The Developing Human . 4th ed. Philadelphia, Pa: WB Saunders Co; 1988:170-206. 5. Sperber GH. Craniofacial Embryology . 4th ed. London, England: Wright; 1989. 6. Nishimura H, Okamoto N. Abnormalities of the face . In: Nishimura H, Okamoto N, eds. Sequential Atlas of Human Malformations . Baltimore, Md: University Park Press; 1976:83. 7. Brauer A. Hereditarer symetrischer Naevus aplasticus bei 38 Personen . Dermatol Wochenschr. 1929;89:1163-1168. 8. Setleis H, Kramer B, Valcarcel M, Einhorn AH. Congenital ectodermal dysplasia of the face . Pediatrics . 1962;32:540-548. 9. Jensen NE. Congenital ectodermal dysplasia of the face . Br J Dermatol. 1971; 84:410-416.Crossref 10. McGeoch AH, Reed WB. Familial focal facial dermal dysplasia . Arch Dermatol. 1973;107:591-596.Crossref 11. Rudolph RI, Schwartz W, Leyden JJ. Bitemporal aplasia cutis congenita: occurrence with other cutaneous anomalies . Arch Dermatol. 1974;110:615-618.Crossref 12. Clark R, Golabi M, Lacassie Y, et al. Expanded phenotype and ethnicity in Setleis syndrome . Am J Med Genet. 1989;34:354-357.Crossref 13. Magid ML, Prendiville J, Esterly NB. Focal facial dermal dysplasia: bitemporal lesions resembling aplasia cutis congenita . J Am Acad Dermatol. 1991;25:389-391.Crossref 14. Kowalski DC, Fenske NA. The focal facial dermal dysplasias: report of a kindred and a proposed new classification . J Am Acad Dermatol. 1992;27:575-582.Crossref 15. Kaplan P, Krantz, I, Mascarenhas M, Tunnessen W, Schulman S. Evidence that the Setleis and Brauer syndromes of focal facial dysplasia are the same entity . Am J Hum Genet. 1995;57:A93. Abstract. 16. Ward K, Moss C. Evidence for genetic homogeneity of Setleis' syndrome and focal facial dermal dysplasia . Br J Dermatol. 1994;130:645-649.Crossref 17. Lindsay EA, Grillo A, Ferrero G, et al. Microphthalmia with linear skin defects (MLS) syndrome: clinical, cytogenetic, and molecular characterization . Am J Med Genet. 1994;49:229-234.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Preauricular Skin Defects: A Consequence of a Persistent Ectodermal Groove

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Publisher
American Medical Association
Copyright
Copyright © 1997 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1997.03890480071010
Publisher site
See Article on Publisher Site

Abstract

Abstract Background: The term aplasia cutis is used to describe congenital localized defects of the skin. This affliction is the end result of various in utero events. Aplasia cutis of the face, although rare, has been associated with numerous dysmorphic features and described under many clinical terms. Observations: We studied 10 patients with oval, atrophic patches distributed in a linear pattern on the preauricular region of the face. Most of the defects were bilateral, and all consistently fell in an oblique line extending from the preauricular region to the angle of the mouth. This line corresponds to the region of fusion between the maxillary and mandibular facial prominences during embryonic development. Conclusions: This type of facial aplasia cutis may be the result of incomplete fusion of the ectodermal groove between the maxillary and mandibular facial prominences. Although other types of facial skin defects may share a similar pathogenic mechanism, they are distinct in that they occur in different regions and may have other abnormal facial features.Arch Dermatol. 1997;133:1551-1554 References 1. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification . J Am Acad Dermatol. 1986;14:646-660.Crossref 2. Drolet BA, Prendiville J, Golden J, Enjolras O, Esterly N. Membranous aplasia cutis with hair collars: congenital absence of the skin or neuroectodermal skin defect? Arch Dermatol. 1995;131:1427-1431.Crossref 3. Gosain AK, Moore FO. Embryology of the head and neck . In: Aston SJ, Beasley RW, Thorne CH, eds. Grabb and Smith's Plastic Surgery . 5th ed. Boston, Mass: Little Brown & Co. In press. 4. Moore KL. The branchial apparatus and the head and neck . In: Moore KL, ed. The Developing Human . 4th ed. Philadelphia, Pa: WB Saunders Co; 1988:170-206. 5. Sperber GH. Craniofacial Embryology . 4th ed. London, England: Wright; 1989. 6. Nishimura H, Okamoto N. Abnormalities of the face . In: Nishimura H, Okamoto N, eds. Sequential Atlas of Human Malformations . Baltimore, Md: University Park Press; 1976:83. 7. Brauer A. Hereditarer symetrischer Naevus aplasticus bei 38 Personen . Dermatol Wochenschr. 1929;89:1163-1168. 8. Setleis H, Kramer B, Valcarcel M, Einhorn AH. Congenital ectodermal dysplasia of the face . Pediatrics . 1962;32:540-548. 9. Jensen NE. Congenital ectodermal dysplasia of the face . Br J Dermatol. 1971; 84:410-416.Crossref 10. McGeoch AH, Reed WB. Familial focal facial dermal dysplasia . Arch Dermatol. 1973;107:591-596.Crossref 11. Rudolph RI, Schwartz W, Leyden JJ. Bitemporal aplasia cutis congenita: occurrence with other cutaneous anomalies . Arch Dermatol. 1974;110:615-618.Crossref 12. Clark R, Golabi M, Lacassie Y, et al. Expanded phenotype and ethnicity in Setleis syndrome . Am J Med Genet. 1989;34:354-357.Crossref 13. Magid ML, Prendiville J, Esterly NB. Focal facial dermal dysplasia: bitemporal lesions resembling aplasia cutis congenita . J Am Acad Dermatol. 1991;25:389-391.Crossref 14. Kowalski DC, Fenske NA. The focal facial dermal dysplasias: report of a kindred and a proposed new classification . J Am Acad Dermatol. 1992;27:575-582.Crossref 15. Kaplan P, Krantz, I, Mascarenhas M, Tunnessen W, Schulman S. Evidence that the Setleis and Brauer syndromes of focal facial dysplasia are the same entity . Am J Hum Genet. 1995;57:A93. Abstract. 16. Ward K, Moss C. Evidence for genetic homogeneity of Setleis' syndrome and focal facial dermal dysplasia . Br J Dermatol. 1994;130:645-649.Crossref 17. Lindsay EA, Grillo A, Ferrero G, et al. Microphthalmia with linear skin defects (MLS) syndrome: clinical, cytogenetic, and molecular characterization . Am J Med Genet. 1994;49:229-234.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Dec 1, 1997

References