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Loose Anagen Syndrome Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 21, 2016
 

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. There may be a history of atopic dermatitis, but the association may be coincidental.[7]

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.

Trichoscopy may show rectangular black granular structures, solitary yellow dots, and major predominance of follicular units with single hairs.[8]

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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 (associations were most likely coincidental):

  • Noonan syndrome[9]
  • Ocular coloboma syndrome[10]
  • Trichorhinophalangeal syndrome
  • Nail-patella syndrome
  • Hypohidrotic ectodermal dysplasia and ectrodactyly-ectodermal dysplasia-clefting syndrome[11]
  • Acquired immunodeficiency syndrome
  • Woolly hair[12]
  • Alopecia areata
  • Loose anagen syndrome with features resembling uncombable hair syndrome[13, 14]
  • 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[15]
  • 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[16]
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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

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

Disclosure: Nothing to disclose.

Coauthor(s)

Supriya Goyal, MD Consulting Dermatologist

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

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

References
  1. Dhurat RP, Deshpande DJ. Loose anagen hair syndrome. Int J Trichology. 2010 Jul. 2(2):96-100. [Medline]. [Full Text].

  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. 2011 Jan. 64(1):129-34. [Medline].

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

  4. Dey V, Thawani M. Loose Anagen Hair Syndrome in Black-Haired Indian Children. Pediatr Dermatol. 2013 Aug 13. [Medline].

  5. Herskovitz I, de Sousa IC, Simon J, Tosti A. Short anagen hair syndrome. Int J Trichology. 2013 Jan. 5(1):45-6. [Medline]. [Full Text].

  6. Agi C, Cohen B. A case of loose anagen syndrome in an African American girl. Pediatr Dermatol. 2015 May-Jun. 32 (3):e128-9. [Medline].

  7. Dey VK, Thawani M. Loose anagen hair syndrome: Is there any association with atopic dermatitis?. Indian Dermatol Online J. 2016 Jan-Feb. 7 (1):56-7. [Medline].

  8. Rakowska A, Zadurska M, Czuwara J, Warszawik-Hendzel O, Kurzeja M, Maj M, et al. Trichoscopy findings in loose anagen hair syndrome: rectangular granular structures and solitary yellow dots. J Dermatol Case Rep. 2015 Mar 31. 9 (1):1-5. [Medline].

  9. 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].

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

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

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

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

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

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

  16. 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. 2003 Apr 30. 118A(3):279-86. [Medline].

  17. Price VH. What looks like alopecia areata is not always alopecia areata. J Investig Dermatol Symp Proc. 2013 Dec. 16(1):S63-4. [Medline].

  18. Zmolikova M, Puchmajerova A, Hecht P, Lebl J, Trkova M, Krepelova A. Coarctation of the aorta in Noonan-like syndrome with loose anagen hair. Am J Med Genet A. 2014 May. 164A(5):1218-21. [Medline].

  19. 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. 2005 Dec. 58(12):1294-8. [Medline].

  20. Chandran NS, Oranje AP. Minoxidil 5% solution for topical treatment of loose anagen hair syndrome. Pediatr Dermatol. 2014 May-Jun. 31(3):389-90. [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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