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CASE REPORT
Year : 2020  |  Volume : 21  |  Issue : 4  |  Page : 332-334

Uncombable hair syndrome with loose anagen syndrome: A rare association


Department of Dermatology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

Date of Submission22-Oct-2018
Date of Decision22-Feb-2019
Date of Acceptance26-Apr-2020
Date of Web Publication30-Sep-2020

Correspondence Address:
Dr. Mohammad Adil
B-9, Rizvi Apartments, Medical Road, Aligarh - 202 002, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_128_18

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  Abstract 


Uncombable hair syndrome presents with dry, coarse, silvery hairs that stand straight from the scalp surface. Loose anagen syndrome is characterized by easy pluckability of the hair due to impaired adhesion between the cuticle of the inner root sheath and the cuticle of the hair shaft. We report the case of a 7-year-old female who presented with features of both these conditions. She had difficulty in combing hair for 4 years. Her hairs did not grow and were dry and frizzy. Hairs were easily pluckable, and microscopy showed predominantly dystrophic anagen hair, with the shaft having undulations and longitudinal groove.

Keywords: Loose anagen syndrome, spun glass hair, uncombable hair syndrome


How to cite this article:
Agrawal D, Amin SS, Adil M, Priya A. Uncombable hair syndrome with loose anagen syndrome: A rare association. Indian J Paediatr Dermatol 2020;21:332-4

How to cite this URL:
Agrawal D, Amin SS, Adil M, Priya A. Uncombable hair syndrome with loose anagen syndrome: A rare association. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Oct 19];21:332-4. Available from: https://www.ijpd.in/text.asp?2020/21/4/332/296845




  Introduction Top


Uncombable hair syndrome (UHS) is a rare hair shaft abnormality characterized by difficulty in combing hairs.[1] Hair shaft is triangular on cross section with or without a groove on one side.[2] It is not associated with short hair. Loose anagen syndrome, on the other hand, is a hair anchorage abnormality leading to excessive and painless loss of anagen hairs.[3] Herein, we describe a patient presenting with the two conditions simultaneously.


  Case Report Top


A 7-year-old female born to nonconsanguineous parents presented with a chief complaint of difficulty in combing hairs for 4 years. The patient's mother first noticed difficult to comb dry and frizzy hairs at the age of 2.5 years when the entire scalp of the baby was shaved bald as a part of religious ceremony. She also noticed that new hairs did not grow long, and the patient had not had a haircut since then. The patient's developmental milestones were not delayed, and there was no history suggestive of physical or mental retardation. There was no history of similar complaint in any family member.

Examination of the scalp revealed that scalp hairs were black in color with a glistening silvery sheen, were dry and frizzy, and stood out from the scalp surface [Figure 1]. There was no sign of scarring, inflammation, or scaling on the scalp. Hairs over the eyebrows, eyelashes, and over rest of the body were normal. Teeth, nails, palms, and soles were normal. There were no ocular, cardiac, neurologic, or skeletal defects. Routine blood investigations, urine routine and microscopic examination, and electrocardiogram were within normal limits.
Figure 1: Dry, frizzy hairs which stand from the scalp surface

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Hair pull test revealed painless and easy extraction of more than ten hairs. Trichogram showed a predominance of dystrophic anagen hairs (>80%) devoid of the outer and inner root sheath (IRS). The proximal segment of the hair shaft closest to the root appeared distorted and twisted, with the characteristic ruffling of the cuticle described as the “floppy sock” appearance [Figure 2]a. Some of the hair bulbs were positioned at an acute angle to the shaft, resembling mouse tails [Figure 2]b. Only one telogen hair was detected. Furthermore, there was difficulty in focusing more than one hair in a single field.
Figure 2: (a) Distorted proximal hair shaft with ruffled cuticle. (b) Hair bulb positioned at an acute angle to the shaft giving a mouse tail appearance

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Examination of the hair shaft under light microscopy revealed light brown pigmentation [Figure 3]a, undulations in the hair shaft, variation in hair shaft diameter, and longitudinal grooves along the edge of hair [Figure 3]b.
Figure 3: (a) Light brown pigmentation of the hair shaft. (b) Undulations in the hair shaft (red arrow) and longitudinal groove along the hair shaft (yellow arrow) and variation in hair shaft diameter

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Scanning electron microscopy was not done due to lack of availability. A diagnosis of UHS with loose anagen syndrome was made, and the mother was counseled about the improvement of disease with age.


  Discussion Top


Loose anagen hair syndrome (LAS) was initially reported in literature in 1986 by Nodl and later by Zaun under the name of “syndrome of loosely attached hairs in childhood.” The term LAS was coined by Price and Gummer. The condition most frequently involves young girls in the age group of 2–6 years presenting with the complaints of lusterless hairs and nongrowing of hairs. It is often misdiagnosed in boys because of short hairs. LAS mainly affects light-colored hairs, although affected patients with dark brown hairs have been reported. It has been reported to be associated with hereditary or developmental disorders. LAS results from substitution mutation in genes encoding cytokeratin K6hf of the companion layer or in genes of IRS, leading to premature keratinization of the IRS that produces an impaired adhesion between the cuticle of the IRS and the cuticle of the hair shaft.[3]

Hair pull test is considered to be positive for LAS if more than ten hairs are extracted out easily and painlessly as compared to the usual one or two hairs in normal individuals. On trichogram, more than 70% of the hairs are dystrophic anagen resembling a floppy sock. Hair bulbs of some of the hairs are at an acute angle resembling mouse tail. The only characteristic finding under scanning electron microscopy is a premature keratinization of the cells of the Henle and Huxley layers of the IRS. Other findings include rippling of the hair cuticle of the proximal hair shaft, triangular or flattened shape, longitudinal grooves, and torsions.[3]

UHS was first described in 1973 as cheveux incoiffable by Dupre and as spun glass hair by Stroud and Mehregan.[1] It is characterized by dry, coarse, frizzy, silvery hairs which stand straight up from the scalp and cannot be combed.[4] More than 50% of the hairs are involved, but the quantity of hairs remains normal. The hairs appear to be glistening because of grooved and flattened hair shaft surfaces. The hairs are slow growing but not fragile. It presents at 3 months to 12 years of age and improves spontaneously before adolescence and has been found to be associated with ectodermal dysplasia and loose anagen syndrome.[5] It has been postulated that it results from mutations in genes such as PADI3, TGM3, and TCHH, which helps provide proper instructions that help in the maintenance of the structure of the hair shaft and gives it a cylindrical shape. As a result of abnormal shape, the hairs tend to not lie flat and appear glistening and unmanageable.[6]

The hair shaft abnormalities seen under light microscopy are light pigmented hairs and very shallow grooves or flattened surfaces that lack periodicity. Cross section of the hairs shows a triangular to reniform to heart-like shape contrasting with a round-to-oval shape in cross section of normal hair.[1]

In 1996, Boyer et al. reported an instance of loose anagen syndrome mimicking uncombable hair. Chapalain et al. described 17 cases of loose anagen syndrome, of which four children had dry, rough, and messy hair, which were difficult to manage.[7] Lee et al. reported another case having features of both of these syndromes and proposed that UHS-like picture with spun glass hair and extensive grooving represents the extreme end of hair shaft abnormality that may be seen in LAS.[8] Another report from India described the association of LAS with wooly hair phenotype in a 2-year-old girl.[9] Biotin has been hypothesized to strengthen hairs by altering hair matrix and makes the hairs more plaint and has been used to manage uncombable hairs.[10] Topical minoxidil has been reported to be a promising treatment of loose anagen syndrome and probably acts by increasing local vascular supply, prolonging keratinocyte lifespan, and prolonging the anagen phase.[3]

Our patient had clinical and microscopic features of loose anagen syndrome and UHS. More studies focusing on biochemical and genetic analysis are needed to ascertain if the two conditions are separate entities or unruly hairs are a manifestation of loose anagen syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for their ward's images and other clinical information to be reported in the journal. The patient's parents understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rieubland C, de Viragh PA, Addor MC. Uncombable hair syndrome: A clinical report. Eur J Med Genet 2007;50:309-14.  Back to cited text no. 1
    
2.
Hicks J, Metry DW, Barrish J, Levy M. Uncombable hair (cheveux incoiffables, pili trianguli et canaliculi) syndrome: Brief review and role of scanning electron microscopy in diagnosis. Ultrastruct Pathol 2001;25:99-103.  Back to cited text no. 2
    
3.
Dhurat RP, Deshpande DJ. Loose anagen hair syndrome. Int J Trichol 2010;2:96.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Sprecher E. Inherited hair disorders. In: GriffithsC, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. West Sussex (UK): Wiley Blackwell Publishing; 2016. p. 68, 22.  Back to cited text no. 4
    
5.
Swamy SS, Ravikumar BC, Vinay KN, Yashovardhana DP, Aggarwal A. Uncombable hair syndrome with a woolly hair nevus. Indian J Dermatol Venereol Leprol 2017;83:87-8.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Basmanav FB, Cau L, Tafazzoli A, Mechin MC, Wolf S, Romano MT, et al. Mutations in three genes encoding proteins involved in hair shaft formation cause uncombable hair syndrome. Am J Hum Gen 2016;99:1292-304.  Back to cited text no. 6
    
7.
Chapalain V, Winter H, Langbein L, Le Roy JM, Labreze C, Nikolic M, et al. Is the loose anagen hair syndrome a keratin disorder? A clinical and molecular study. Arch Dermatol 2002;138:501-6.  Back to cited text no. 7
    
8.
Lee AJ, Maino KL, Cohen B, Sperling L. A girl with loose anagen hair syndrome and uncombable, spun-glass hair. Pediatr Dermatol 2005;22:230-3.  Back to cited text no. 8
    
9.
Arshdeep, Mahajan R, De D, Handa S. Loose anagen hair syndrome with diffuse woolly hair phenotype: A rare association. Indian J Paediatr Dermatol 2016;17:142-4.  Back to cited text no. 9
    
10.
Stanley WB, Shelley ED. Uncombable hair syndrome: Observations on response to biotin and occurrence in siblings with ectodermal dysplasia. J Am Acad Dermatol 1985;13:97-102.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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