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'Membranous Aplasia Cutis' With Hair Collars: Congenital Absence of Skin or Neuroectodermal Defect?

'Membranous Aplasia Cutis' With Hair Collars: Congenital Absence of Skin or Neuroectodermal Defect? Abstract Background: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. Observations: We describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects. Conclusions: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.(Arch Dermatol. 1995;131:1427-1431) References 1. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair: the 'hair collar' sign . Arch Dermatol. 1989;125:1253-1256.Crossref 2. Cordon M. Extrait d'une lettre au sujet de trois enfants de la même mère nés avec une partie des extremités deníees de peau . J Med Chir Pharmacie. 1767; 26:556-557. 3. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification . J Am Acad Dermatol. 1986;14:646-660.Crossref 4. Drolet BA, Clowry LA, McTigue MK, Esterly NB. The hair collar sign: a marker for cranial dysraphism . Pediatrics. 1995;96:309-313. 5. Golden JA, Chernoff GF. An intermittent pattern of anterior neural tube closure in humans: evidence from anterior neural tube defects (anencephaly) . Pediatrics. 1995;95:506-510. 6. Van Allen MI, Dagmar KK, Chernoff GF, et al. Evidence for multi-site closure of the neural tube in humans . Am J Med Genet. 1993;47:723-743.Crossref 7. Golden JA, Chernoff GF. Intermittent pattern of neural tube closure in two strains of mice . Teratology . 1993;47:73-80.Crossref 8. MacDonald KB, Juriloff DM, Harris MJ. Developmental studies of neural tube closure in mouse stock with a high incidence of exencephaly . Teratology . 1989; 39:195-213.Crossref 9. Stephan MJ, Smith DW, Ponzi JW, Major MC, Alden ER, Clonel MC. Origin of scalp aplasia cutis . J Pediatr. 1982;101:850-853.Crossref 10. Lever WF. Aplasia cutis . In: Histopathology of the Skin . 7th ed. Philadelphia, Pa: JB Lippincott; 1990:65-102. 11. Demmel U. Clinical aspects of congenital skin defects, I: congenital skin defects on the head of the newborn . Eur J Pediatr. 1975;121:21-50.Crossref 12. McLaurin RL. Parietal cephaloceles . Neurology . 1964;14:764-772.Crossref 13. Tanii T, Hamada T. A variant of encephalomeningocele: heterotopic brain tissue of the scalp . Dermatologica. 1984;169:354-358.Crossref 14. Sibley DA, Cooper PH. Rudimentary meningocele: a variant of 'primary cutaneous meningioma.' J Cutan Pathol. 1989;16:72-80.Crossref 15. Khallouf R, Fetissof F, Machet MC, Stephanov E, Lechrist J, Lorette G. Sequestered meningocele of the scalp: diagnostic value of hair anomalies . Pediatr Dermatol. 1994;11:315-318.Crossref 16. Orkin M, Fisher I. Heterotopic brain tissue (heterotopic neural rest): case report with review of related anomalies . Arch Dermatol. 1966;94:699-707.Crossref 17. Kosnik EJ, Sayer MP. Congenital scalp defects: aplasia cutis congenita . J Neurosurg. 1975;42:32-36.Crossref 18. Ingalls NW. Congenital defects of the scalp, III: studies in the pathology of development . Am J Obstet Gynecol. 1933;25:861-873. 19. Yokota A, Matsukado Y. Congenital midline porencephaly: a new brain malformation associated with scalp anomaly . Childs Brain. 1979;5:380-397. 20. Singman R, Asaikan S, Hotson G, Prose N. Aplasia cutis congenita and arteriovenous fistula . Arch Neurol. 1990;47:1255-1258.Crossref 21. Dellman JW, Oorthuys JWE. Orbital cyst in addition to congenital cerebral and focal dermal malformations: a new entity? Clin Genet. 1981;19:191-198.Crossref 22. Wilson RD, Traverse L, Hall JG, Foldmark CO, Rootman J. Oculocutaneous syndrome associated with scalp anomaly . Childs Brain. 1979;5:380-397. 23. Khoury MJ, Cordero JF, Rasmussen S. Etiologic heterogeneity of neural tube defects: clues from epidemiology . Am J Epidemiol. 1988;115:538-548. 24. Pap GS. Congenital defect of the scalp and skull in three generations of one family . Plast Reconstr Surg. 1970;46:194-196.Crossref 25. Guillen PS, Pichardo AR, Martinez FC. Aplasia cutis congenita . J Am Acad Dermatol. 1985;13:429-433.Crossref 26. Fryne JP, Van den Berghe H. Congenital scalp defects associated with postaxial polydactyly . Hum Genet. 1979;49:217-219.Crossref 27. Abuelo D, Feingold M. Scalp defects in trisomy 13 . Clin Pediatr. 1969;8:416-417.Crossref 28. Campell W. Case of congenital ulcer on the cranium of a fetus, terminating in fatal hemorrhage on the 18th day after birth . Edinb J Med Sci. 1826;2:82-83. 29. Freide RL. Developmental Neuropathy . New York, NY: Springer-Verlag NY Inc; 1989:248-262, 387-394. 30. Rodriguez JI, Garcia M, Morales C, Morillo A, Delicado A. Trisomy 13 syndrome and neural tube defects . Am J Med Genet. 1990;36:513-516.Crossref 31. Fitzsimomons GM, Filshie GM, Hill AS, Kime R. Antenatal diagnosis of trisomy 13 with unexpected increase in alpha-fetoprotein . J Med Genet. 1976;13:400-412.Crossref 32. Guibaud S, Pison H, Simplot A, Boisson C, Germain D. Élévation inhabituelle de l'alpha-foetoprotéine du liquide amniotique et trisomie 13 . J Genet Hum. 1987;35:237-241. 33. Farine D, Maidman J, Rubin S, Chao S. Elevated alpha-fetoprotein in pregnancy complicated by aplasia cutis after exposure to methimazole . Obstet Gynecol. 1988;71:996-997. 34. Bick DP, Balkite MS, Baumgarten JC, Hobbins JC, Mahoney MJ. The association of congenital skin disorders with acetylcholinesterase in amniotic fluid . Prenat Diagn. 1987;7:543-549.Crossref 35. Sakai Y. Neurulation in the mouse: manner and timing of neural tube defects . Anat Rec. 1989;223:194-203.Crossref 36. Tom C, Juriloff DM, Harris MJ. Studies of the effect of retinoic acid on anterior neural tube closure in mice genetically liable to exencephaly . Teratology. 1991;43:27-40.Crossref 37. Golden JA, Chernoff GF. Anterior neural tube closure in the mouse: fuel for disagreement with the classical theory . Clin Res. 1983;31:127A. Abstract. 38. Cunningham BA. Cell adhesion molecules and the regulation of development . Am J Obstet Gynecol. 1991;164:939-948.Crossref 39. Holbrook KA, Smith LT, Kaplan ED, Minami SA, Hebert GP, Underwood RA. Expression of morphogens during human follicle development in vivo and a model for studying follicle morphogens in vitro . J Invest Dermatol. 1993;101: 39S-47S.Crossref 40. Chuong CM. Adhesion molecules (N-CAM and tenascin) in embryonic development and tissue regeneration . J Craniofac Genet Dev Biol. 1990;10:147-161. 41. Chuong CM, Chen H, Jiang T, Chia J. Adhesion molecules in skin development: morphogenesis of feather and hair . Ann N Y Acad Sci. 1991;642:263-280.Crossref 42. Edelman GM. Cell adhesion molecules in the regulation of animal form and tissue pattern . Ann Rev Cell Biol. 1986;2:81-116.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

'Membranous Aplasia Cutis' With Hair Collars: Congenital Absence of Skin or Neuroectodermal Defect?

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Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1995.01690240091015
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Abstract

Abstract Background: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. Observations: We describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects. Conclusions: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.(Arch Dermatol. 1995;131:1427-1431) References 1. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair: the 'hair collar' sign . Arch Dermatol. 1989;125:1253-1256.Crossref 2. Cordon M. Extrait d'une lettre au sujet de trois enfants de la même mère nés avec une partie des extremités deníees de peau . J Med Chir Pharmacie. 1767; 26:556-557. 3. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification . J Am Acad Dermatol. 1986;14:646-660.Crossref 4. Drolet BA, Clowry LA, McTigue MK, Esterly NB. The hair collar sign: a marker for cranial dysraphism . Pediatrics. 1995;96:309-313. 5. Golden JA, Chernoff GF. An intermittent pattern of anterior neural tube closure in humans: evidence from anterior neural tube defects (anencephaly) . Pediatrics. 1995;95:506-510. 6. Van Allen MI, Dagmar KK, Chernoff GF, et al. Evidence for multi-site closure of the neural tube in humans . Am J Med Genet. 1993;47:723-743.Crossref 7. Golden JA, Chernoff GF. Intermittent pattern of neural tube closure in two strains of mice . Teratology . 1993;47:73-80.Crossref 8. MacDonald KB, Juriloff DM, Harris MJ. Developmental studies of neural tube closure in mouse stock with a high incidence of exencephaly . Teratology . 1989; 39:195-213.Crossref 9. Stephan MJ, Smith DW, Ponzi JW, Major MC, Alden ER, Clonel MC. Origin of scalp aplasia cutis . J Pediatr. 1982;101:850-853.Crossref 10. Lever WF. Aplasia cutis . In: Histopathology of the Skin . 7th ed. Philadelphia, Pa: JB Lippincott; 1990:65-102. 11. Demmel U. Clinical aspects of congenital skin defects, I: congenital skin defects on the head of the newborn . Eur J Pediatr. 1975;121:21-50.Crossref 12. McLaurin RL. Parietal cephaloceles . Neurology . 1964;14:764-772.Crossref 13. Tanii T, Hamada T. A variant of encephalomeningocele: heterotopic brain tissue of the scalp . Dermatologica. 1984;169:354-358.Crossref 14. Sibley DA, Cooper PH. Rudimentary meningocele: a variant of 'primary cutaneous meningioma.' J Cutan Pathol. 1989;16:72-80.Crossref 15. Khallouf R, Fetissof F, Machet MC, Stephanov E, Lechrist J, Lorette G. Sequestered meningocele of the scalp: diagnostic value of hair anomalies . Pediatr Dermatol. 1994;11:315-318.Crossref 16. Orkin M, Fisher I. Heterotopic brain tissue (heterotopic neural rest): case report with review of related anomalies . Arch Dermatol. 1966;94:699-707.Crossref 17. Kosnik EJ, Sayer MP. Congenital scalp defects: aplasia cutis congenita . J Neurosurg. 1975;42:32-36.Crossref 18. Ingalls NW. Congenital defects of the scalp, III: studies in the pathology of development . Am J Obstet Gynecol. 1933;25:861-873. 19. Yokota A, Matsukado Y. Congenital midline porencephaly: a new brain malformation associated with scalp anomaly . Childs Brain. 1979;5:380-397. 20. Singman R, Asaikan S, Hotson G, Prose N. Aplasia cutis congenita and arteriovenous fistula . Arch Neurol. 1990;47:1255-1258.Crossref 21. Dellman JW, Oorthuys JWE. Orbital cyst in addition to congenital cerebral and focal dermal malformations: a new entity? Clin Genet. 1981;19:191-198.Crossref 22. Wilson RD, Traverse L, Hall JG, Foldmark CO, Rootman J. Oculocutaneous syndrome associated with scalp anomaly . Childs Brain. 1979;5:380-397. 23. Khoury MJ, Cordero JF, Rasmussen S. Etiologic heterogeneity of neural tube defects: clues from epidemiology . Am J Epidemiol. 1988;115:538-548. 24. Pap GS. Congenital defect of the scalp and skull in three generations of one family . Plast Reconstr Surg. 1970;46:194-196.Crossref 25. Guillen PS, Pichardo AR, Martinez FC. Aplasia cutis congenita . J Am Acad Dermatol. 1985;13:429-433.Crossref 26. Fryne JP, Van den Berghe H. Congenital scalp defects associated with postaxial polydactyly . Hum Genet. 1979;49:217-219.Crossref 27. Abuelo D, Feingold M. Scalp defects in trisomy 13 . Clin Pediatr. 1969;8:416-417.Crossref 28. Campell W. Case of congenital ulcer on the cranium of a fetus, terminating in fatal hemorrhage on the 18th day after birth . Edinb J Med Sci. 1826;2:82-83. 29. Freide RL. Developmental Neuropathy . New York, NY: Springer-Verlag NY Inc; 1989:248-262, 387-394. 30. Rodriguez JI, Garcia M, Morales C, Morillo A, Delicado A. Trisomy 13 syndrome and neural tube defects . Am J Med Genet. 1990;36:513-516.Crossref 31. Fitzsimomons GM, Filshie GM, Hill AS, Kime R. Antenatal diagnosis of trisomy 13 with unexpected increase in alpha-fetoprotein . J Med Genet. 1976;13:400-412.Crossref 32. Guibaud S, Pison H, Simplot A, Boisson C, Germain D. Élévation inhabituelle de l'alpha-foetoprotéine du liquide amniotique et trisomie 13 . J Genet Hum. 1987;35:237-241. 33. Farine D, Maidman J, Rubin S, Chao S. Elevated alpha-fetoprotein in pregnancy complicated by aplasia cutis after exposure to methimazole . Obstet Gynecol. 1988;71:996-997. 34. Bick DP, Balkite MS, Baumgarten JC, Hobbins JC, Mahoney MJ. The association of congenital skin disorders with acetylcholinesterase in amniotic fluid . Prenat Diagn. 1987;7:543-549.Crossref 35. Sakai Y. Neurulation in the mouse: manner and timing of neural tube defects . Anat Rec. 1989;223:194-203.Crossref 36. Tom C, Juriloff DM, Harris MJ. Studies of the effect of retinoic acid on anterior neural tube closure in mice genetically liable to exencephaly . Teratology. 1991;43:27-40.Crossref 37. Golden JA, Chernoff GF. Anterior neural tube closure in the mouse: fuel for disagreement with the classical theory . Clin Res. 1983;31:127A. Abstract. 38. Cunningham BA. Cell adhesion molecules and the regulation of development . Am J Obstet Gynecol. 1991;164:939-948.Crossref 39. Holbrook KA, Smith LT, Kaplan ED, Minami SA, Hebert GP, Underwood RA. Expression of morphogens during human follicle development in vivo and a model for studying follicle morphogens in vitro . J Invest Dermatol. 1993;101: 39S-47S.Crossref 40. Chuong CM. Adhesion molecules (N-CAM and tenascin) in embryonic development and tissue regeneration . J Craniofac Genet Dev Biol. 1990;10:147-161. 41. Chuong CM, Chen H, Jiang T, Chia J. Adhesion molecules in skin development: morphogenesis of feather and hair . Ann N Y Acad Sci. 1991;642:263-280.Crossref 42. Edelman GM. Cell adhesion molecules in the regulation of animal form and tissue pattern . Ann Rev Cell Biol. 1986;2:81-116.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Dec 1, 1995

References