eMedicine Specialties > Dermatology > Diseases of the Adnexa
Loose Anagen Syndrome
Updated: Sep 23, 2008
Introduction
Background
Loose anagen syndrome was first described in 1984. It is a hair disorder characterized by anagen hairs of abnormal morphology that are easily and painlessly pulled or plucked from the scalp. Hair is thinned in appearance and typically does not grow beyond the nape of the neck.
Pathophysiology
The precise pathogenesis of loose anagen syndrome is not known, but theories postulating an abnormality in the hair's anchoring mechanism predominate.
The inner root sheath is thought to play an integral role in anchoring the hair shaft within the follicle. In loose anagen syndrome, mutations in genes coding for cytokeratins have been identified in some cases and are thought to result in abnormal keratinization of the inner root sheath. This faulty keratinization leads to impaired adherence of the deformed hair shafts to their follicles. The impaired attachment may result in premature cessation of the anagen phase and account for reduced hair length. One author has also reported reduced follicle size due to delayed maturation. This may be responsible for sparse hair growth.
Frequency
United States
The prevalence of loose anagen syndrome is unknown. Although recently described, loose anagen syndrome may actually be rather common. It is undoubtedly often misdiagnosed as alopecia areata or trichotillomania.1 It has likely been both underdiagnosed and underreported to date.
Mortality/Morbidity
The diffuse hair loss that occurs in loose anagen syndrome often raises considerable concern among patients, parents, and clinicians. Although no treatment is currently available, the condition is of cosmetic significance only, and symptoms generally improve over time.
Race
Loose anagen syndrome has been reported only in white persons.
Sex
The syndrome is more common in females than in males.
Age
The classic patient with loose anagen syndrome is a girl aged 2-5 years with blonde hair; however, cases of loose anagen syndrome in boys, in adults, and in individuals with dark hair have also been reported.
Clinical
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.
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 Media File 1).
- 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.
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 syndrome2
- Ocular coloboma syndrome3
- Trichorhinophalangeal syndrome
- Nail-patella syndrome
- Hypohidrotic ectodermal dysplasia and ectrodactyly-ectodermal dysplasia-clefting syndrome4
- Acquired immunodeficiency syndrome
- Woolly hair5
- Alopecia areata
- Loose anagen syndrome with features resembling uncombable hair syndrome6,7
- Colobomas and dysmorphic features including low-set ears, hypertelorism, left microphthalmia, frontal bossing, a thin upper lip, a simple philtrum, and slight left facial hypoplasia8
- 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 pigmentation9
More on Loose Anagen Syndrome |
Overview: Loose Anagen Syndrome |
| Differential Diagnoses & Workup: Loose Anagen Syndrome |
| Treatment & Medication: Loose Anagen Syndrome |
| Follow-up: Loose Anagen Syndrome |
| Multimedia: Loose Anagen Syndrome |
| References |
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References
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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].
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].
Azon-Masoliver A, Ferrando J. Loose anagen hair in hypohidrotic ectodermal dysplasia. Pediatr Dermatol. Jan-Feb 1996;13(1):29-32. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
Chong AH, Sinclair R. Loose anagen syndrome: a prospective study of three families. Australas J Dermatol. May 2002;43(2):120-4. [Medline].
Hamm H, Traupe H. Loose anagen hair of childhood: the phenomenon of easily pluckable hair. J Am Acad Dermatol. Feb 1989;20(2 Pt 1):242-8. [Medline].
Haskett M. Loose anagen syndrome. Australas J Dermatol. Feb 1995;36(1):35-6. [Medline].
Khadir K, Habibeddine S, Azzouzi S, Lakhdar H, Van Neste D. [Loose anagen hair syndrome: a familial case with fetal hair in meconium]. Ann Dermatol Venereol. Jan 2001;128(1):52-4. [Medline].
Li VW, Baden HP, Kvedar JC. Loose anagen syndrome and loose anagen hair. Dermatol Clin. Oct 1996;14(4):745-51. [Medline].
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].
O'Donnell BP, Sperling LC, James WD. Loose anagen hair syndrome. Int J Dermatol. Feb 1992;31(2):107-9. [Medline].
Pride HB, Tunnessen WW Jr. Picture of the month. Loose anagen syndrome. Arch Pediatr Adolesc Med. Jul 1995;149(7):819-20. [Medline].
Sinclair R, Cargnello J, Chow CW. Loose anagen syndrome. Exp Dermatol. Aug 1999;8(4):297-8. [Medline].
Skelsey MA, Price VH. Noninfectious hair disorders in children. Curr Opin Pediatr. Aug 1996;8(4):378-80. [Medline].
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].
Sullivan JR, Kossard S. Acquired scalp alopecia. Part I: A review. Australas J Dermatol. Nov 1998;39(4):207-19; quiz 220-1. [Medline].
Thomas L, Robart S, Balme B, Moulin G. [Loose anagen hair syndrome]. Ann Dermatol Venereol. 1993;120(8):535-7. [Medline].
Tosti A, Peluso AM, Misciali C, Venturo N, Patrizi A, Fanti PA. Loose anagen hair. Arch Dermatol. Sep 1997;133(9):1089-93. [Medline].
Tosti A, Piraccini BM. Loose anagen hair syndrome and loose anagen hair. Arch Dermatol. Apr 2002;138(4):521-2. [Medline].
Further Reading
Keywords
loose anagen hair syndrome, short anagen syndrome, loose anagen hair of childhood, easily pluckable hair
Overview: Loose Anagen Syndrome