|Year : 2015 | Volume
| Issue : 1 | Page : 36-38
Loose anagen hair syndrome in a child
Urmi Khanna, Paschal D'Souza
Department of Dermatology, ESI PGIMSR, Basaidarapur, New Delhi, India
|Date of Web Publication||16-Jan-2015|
Department of Dermatology, ESI PGIMSR, Basaidarapur, New Delhi
Source of Support: None, Conflict of Interest: None
Loose anagen hair syndrome (LAHS) is due to a defective anchorage of the hair shaft to the follicle, resulting in retarded hair growth and diffuse or patchy alopecia. LAHS usually presents as diffuse alopecia in blonde girls, occurring as isolated cases but rarely in association with hereditary or developmental disorders. We report this case because of the unique presentation of LAHS in a dark-skinned male toddler as patchy alopecia in association with developmental delay.
Keywords: Alopecia, loose anagen, noncicatricial
|How to cite this article:|
Khanna U, D'Souza P. Loose anagen hair syndrome in a child. Indian J Paediatr Dermatol 2015;16:36-8
| Introduction|| |
Loose anagen hair syndrome (LAHS) is due to a defective anchorage of the hair shaft to the follicle, resulting in retarded hair growth and diffuse or patchy alopecia. LAHS is predominantly seen in young girls between 2 and 6 years, being underdiagnosed in boys because of hairstyle differences.  We present a case of LAHS presenting as patchy hypotrichosis in a child.
| Case report|| |
A 3-year-old male child presented for evaluation of reduced hair density over a few areas of the scalp. The parents reported inability of hair to grow in length with the child requiring only a single haircut since birth. There was no history of hair problems in parents or siblings. Also, there was no history of preceding stressor or repeated pulling of hair by the child or any other individual. Scalp examination revealed diffuse thin lusterless brown hair with irregular patches of hypotrichosis over the fronto-temporal and occipital area. The patch over the occipital area was most distinct showing hairshafts of variable length [Figure 1]. The child had dolicocephaly, evidence of developmental delay and enamel hypoplasia of lower canines. Eyebrows, eyelashes, nails, and eyes were normal. Clinical differential diagnosis of trichotillomania, alopecia areata, and telogen effluvium were made. Hair pull test yielded painlessly pluckable predominantly anagen hair >70% of which on light microscopy were devoid of root sheaths with 'floppy sock appearance' of the cuticle [Figure 2]. Radiological and laboratory investigations were normal. A biopsy was taken from the occipital scalp. Hematoxylin and eosin staining of the tissue specimen revealed evidence of trichomalacia and follicular plugging [Figure 3]. However, there was no evidence of any inflammatory infiltrate. The parents were counseled about the spontaneous resolution of the condition and thus, no treatment was administered. Follow-up at 5 months showed a marked improvement in the hair density especially over the occiput [Figure 4].
|Figure 1: Irregular patchy alopecia over the occipital area of scalp with hair shafts of varying lengths|
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|Figure 2: Microscopic examination shows the "floppy sock" appearance of the hair shaft|
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|Figure 3: Hematoxylin and eosin staining (×400) of the biopsy specimen shows the misshapen follicles without any perifollicular inflammatory infiltrate|
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|Figure 4: Near complete recovery of the hypotrichosis at 5 months follow-up|
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| Discussion|| |
Loose anagen syndrome (LAS) is a condition that typically begins around the age of 2 years, and is characterized by loosely anchored anagen hairs. It is inherited as an autosomal dominant trait with variable expressivity.  Most authors believe that LAH results from a premature keratinization of the inner root sheath that produces an impaired adhesion between the cuticle of the inner root sheath and the cuticle of the hair shaft.  Historical features include hair, that fails to grow long and rarely, requires trimming, diffuse or patchy thinning with hairs of uneven length and patches of hair loss due to inadvertent hair pulling.  It is clinically heterogeneous and ranges from a full head of hair to areas of diffuse thinning and irregular patchy hair loss, and the texture has been described as sticky, dry, or rough.  On the basis of the predominance of the 3 most important clinical signs (reduced hair length, increased hair shedding, and altered hair texture), Olsen et al. have distinguished patients with three different phenotypes of LAS: (1) Patients with type A LAS have sparse hair that does not grow long, and they often present with patches of dull, unruly hair; (2) patients with type B LAS have diffuse or patchy, unruly hair; and (3) patients with type C LAS have increased hair shedding.  The diagnosis is essentially clinical, with the hallmark feature being the ability to extract anagen hairs easily and painlessly with a simple hair pull test. It is presumed to be due to dis-adhesion or weak adhesion between the hair shaft cuticle and inner root sheath cuticle.  Microscopy of a plucked hair may show ruffling of the cuticle adjacent to the anagen bulb, giving the appearance of a "floppy sock."  In 1997, the following diagnostic criteria were proposed for LAHS: (1) Positive pull test results with painless extraction of at least 10 LAHs; (2) the presence of more than 80% LAH at trichogram.  These criteria, however, are probably too strict and permit diagnosis of only the most severely affected patients. After further experience with these criteria, it is now believed that a negative pull test finding does not exclude the diagnosis of LAS. However, it still believed by some authors that the diagnosis of LAS should not be made if the trichogram does not show at least 70% LAH.  Histological examination shows premature keratinization of the inner root sheath layers of Huxley and Henle. Keratin K6irs is an inner root sheath keratin proposed as a protein that might control manifestations of the disorder.  The differential diagnosis of LAHS in a child includes trichotillomania, alopecia areata, and telogen effluvium, broadly all of which can be differentiated based on an increased fraction telogen hair in the trichogram besides their pattern of hair loss and clinical course.  LAHS usually improves spontaneously within a few years. When the child grows up, the hair becomes longer, denser, and darker.  Some authorities have had good results with 5% topical minoxidil, although it is not commonly employed and is not of clear value.  A diagnosis of LAHS may be missed if physicians are unaware of this clinical entity. Thus, it is suggested that while examining a child presenting with noncicatricial patchy hypotrichosis a differential diagnosis of LAHS should be borne in mind, and besides a detailed history and thorough examination, a hair pull test should also be performed to ascertain the diagnosis. LAHS usually presents as diffuse alopecia in blonde girls, occurring as isolated cases but rarely in association with hereditary or developmental disorders. We report this case because of the unique presentation of LAHS in a dark-skinned male toddler as patchy alopecia in association with developmental delay.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]