Loose Anagen Syndrome Clinical Presentation

  • Author: Supriya Goyal, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 22, 2011
 

History

  • Parents often report that the child's hair is thinning and that haircuts are never needed or are needed only very infrequently.
  • Parents may have noticed that hair traction, either accidental during playing or intentional, yields clumps of painlessly removed hair.
  • Many parents complain that hair is unmanageable, lusterless, dry, dull, or matted.
  • Parents or siblings occasionally have a history of similar symptoms.
  • Children who are affected are healthy and free from underlying nutritional deficiencies or other illnesses.
  • Growth and development are normal.
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Physical

  • Physical examination reveals sparse growth of thin, fine hair and diffuse or patchy alopecia without inflammation or scarring.
  • Gentle traction results in hair that is painlessly removed; however, hair is not fragile or easily breakable.
  • Hair may be of varying lengths and may have an unkempt, lackluster appearance.
  • In particular, hair overlying the occiput tends to be rough or sticky and does not lie flat (see following image).Thinning, dull, lackluster blonde hair with mattinThinning, dull, lackluster blonde hair with matting over the occipital region.
  • No scalp inflammation or scarring is present.
  • Eyebrows, eyelashes, and body hair are rarely involved.
  • Other structures of ectodermal origin (eg, skin, teeth, nails) are not affected.
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Causes

Although its occurrence is typically sporadic, familial cases of loose anagen syndrome have been observed. Inheritance appears to be in an autosomal dominant pattern with variable penetrance. Loose anagen syndrome has not been consistently associated with any other disorder; however, individual cases associated with the following syndromes have been reported. These associations were most likely coincidental.

  • Noonan syndrome[4]
  • Ocular coloboma syndrome[5]
  • Trichorhinophalangeal syndrome
  • Nail-patella syndrome
  • Hypohidrotic ectodermal dysplasia and ectrodactyly-ectodermal dysplasia-clefting syndrome[6]
  • Acquired immunodeficiency syndrome
  • Woolly hair[7]
  • Alopecia areata
  • Loose anagen syndrome with features resembling uncombable hair syndrome[8, 9]
  • Colobomas and dysmorphic features including low-set ears, hypertelorism, left microphthalmia, frontal bossing, a thin upper lip, a simple philtrum, and slight left facial hypoplasia[10]
  • Noonan-like syndrome characterized by short stature, a distinctive facial phenotype, macrocephaly, enlarged cerebral spinal fluid spaces, a short neck with redundant skin, severe growth hormone deficiency, mild psychomotor delay with attention deficit/hyperactivity disorder, and increased skin pigmentation[11]
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Contributor Information and Disclosures
Author

Supriya Goyal, MD  Consulting Dermatologist

Supriya Goyal, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Leonard Sperling, MD  Chair, Professor, Department of Dermatology, Uniformed Services University of the Health Sciences

Leonard Sperling, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Paul Krusinski, MD  Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
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  2. Mirmirani P, Uno H, Price VH. Abnormal inner root sheath of the hair follicle in the loose anagen hair syndrome: an ultrastructural study. J Am Acad Dermatol. Jan 2011;64(1):129-34. [Medline].

  3. Thai KE, Sinclair RD. Loose anagen syndrome as a severity factor for trichotillomania. Br J Dermatol. Oct 2002;147(4):789-92. [Medline].

  4. Tosti A, Misciali C, Borrello P, Fanti PA, Bardazzi F, Patrizi A. Loose anagen hair in a child with Noonan's syndrome. Dermatologica. 1991;182(4):247-9. [Medline].

  5. Murphy MF, McGinnity FG, Allen GE. New familial association between ocular coloboma and loose anagen syndrome. Clin Genet. Apr 1995;47(4):214-6. [Medline].

  6. Azon-Masoliver A, Ferrando J. Loose anagen hair in hypohidrotic ectodermal dysplasia. Pediatr Dermatol. Jan-Feb 1996;13(1):29-32. [Medline].

  7. García-Hernández MJ, Price VH, Camacho FM. Woolly hair associated with loose anagen hair. Acta Derm Venereol. Sep-Oct 2000;80(5):388-9. [Medline].

  8. Boyer JD, Cobb MW, Sperling LC, Rushin JM. Loose anagen hair syndrome mimicking the uncombable hair syndrome. Cutis. Feb 1996;57(2):111-2. [Medline].

  9. Lee AJ, Maino KL, Cohen B, Sperling L. A girl with loose anagen hair syndrome and uncombable, spun-glass hair. Pediatr Dermatol. May-Jun 2005;22(3):230-3. [Medline].

  10. Hansen LK, Brandrup F, Clemmensen O. Loose anagen hair syndrome associated with colobomas and dysmorphic features. Clin Dysmorphol. Jan 2004;13(1):31-2. [Medline].

  11. Mazzanti L, Cacciari E, Cicognani A, Bergamaschi R, Scarano E, Forabosco A. Noonan-like syndrome with loose anagen hair: a new syndrome?. Am J Med Genet A. Apr 30 2003;118A(3):279-86. [Medline].

  12. Baden HP, Kvedar JC, Magro CM. Loose anagen hair as a cause of hereditary hair loss in children. Arch Dermatol. Oct 1992;128(10):1349-53. [Medline].

  13. Chapalain V, Winter H, Langbein L, Le Roy JM, Labrèze C, Nikolic M, et al. Is the loose anagen hair syndrome a keratin disorder? A clinical and molecular study. Arch Dermatol. Apr 2002;138(4):501-6. [Medline].

  14. Chapman DM, Miller RA. An objective measurement of the anchoring strength of anagen hair in an adult with the loose anagen hair syndrome. J Cutan Pathol. Jun 1996;23(3):288-92. [Medline].

  15. Chong AH, Sinclair R. Loose anagen syndrome: a prospective study of three families. Australas J Dermatol. May 2002;43(2):120-4. [Medline].

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  17. Haskett M. Loose anagen syndrome. Australas J Dermatol. Feb 1995;36(1):35-6. [Medline].

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  19. Li VW, Baden HP, Kvedar JC. Loose anagen syndrome and loose anagen hair. Dermatol Clin. Oct 1996;14(4):745-51. [Medline].

  20. Martínez JA, Velasco M, Vilata JJ, Quecedo E, Aliaga A. Loose anagen syndrome: a new case. Acta Derm Venereol. Nov 1994;74(6):473. [Medline].

  21. O'Donnell BP, Sperling LC, James WD. Loose anagen hair syndrome. Int J Dermatol. Feb 1992;31(2):107-9. [Medline].

  22. Pride HB, Tunnessen WW Jr. Picture of the month. Loose anagen syndrome. Arch Pediatr Adolesc Med. Jul 1995;149(7):819-20. [Medline].

  23. Sinclair R, Cargnello J, Chow CW. Loose anagen syndrome. Exp Dermatol. Aug 1999;8(4):297-8. [Medline].

  24. Skelsey MA, Price VH. Noninfectious hair disorders in children. Curr Opin Pediatr. Aug 1996;8(4):378-80. [Medline].

  25. Smith VV, Anderson G, Malone M, Sebire NJ. Light microscopic examination of scalp hair samples as an aid in the diagnosis of paediatric disorders: retrospective review of more than 300 cases from a single centre. J Clin Pathol. Dec 2005;58(12):1294-8. [Medline].

  26. Sullivan JR, Kossard S. Acquired scalp alopecia. Part I: A review. Australas J Dermatol. Nov 1998;39(4):207-19; quiz 220-1. [Medline].

  27. Thomas L, Robart S, Balme B, Moulin G. [Loose anagen hair syndrome]. Ann Dermatol Venereol. 1993;120(8):535-7. [Medline].

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  29. Tosti A, Piraccini BM. Loose anagen hair syndrome and loose anagen hair. Arch Dermatol. Apr 2002;138(4):521-2. [Medline].

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Thinning, dull, lackluster blonde hair with matting over the occipital region.
Microscopic view of easily plucked hair demonstrating a distorted anagen bulb lacking inner and outer root sheaths, and a ruffled, sawtooth, baggy-stocking appearance of the cuticle (original magnification X100).
 
 
 
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