Loose Anagen Hair Syndrome in Black-HairedIndian Children

Vivek Dey, M.D., and Manasi Thawani, M.D.

Department of Dermatology, Venereology and Leprosy, People’s College of Medical Sciences & Research Centre,Bhopal, MP, India

Abstract: Loose anagen hair syndrome (LAHS) is an uncommonlyreported autosomal dominant hair disorder with incomplete penetrancethat primarily affects children but is occasionally seen in adults. LAHS ischaracterized by the ability to easily and painlessly extract unsheathedanagen hairs from the scalp with gentle traction. The hair is sparse anddoes not grow long. Usually the hairs are not fragile and do not have areasof breakage. Initially it was considered a rare, sporadic condition foundpredominantly in young white girls with blonde hair. Because autosomaldominant inheritance has been reported, it was suspected that thecondition might be equally common in boys but was probably under-diagnosed.

Reports of loose anagen hair syndrome (LAHS) indark-haired children are lacking. The first report ofLAHS in dark-skinned children was reported fromEgypt in 2009 (1). There is a paucity of reports ofLAHS in dark-skinned individuals, especially fromAsian and African countries.

PATIENTS AND METHODS

Children younger than 12 years old attending ouroutpatient clinic with complaints of increased hairloss, slow hair growth, or sparseness of hair within aperiod of 2 years from October 2010 to October 2012were included for study. Appropriate institutionalethical committee approval and informed consentfrom patients were obtained. A detailed history,including family history, was taken and a detailedclinical examination, hair pull test, and hair pluck

trichogram were performed. Other possible causes ofdiffuse or patchy nonscarring hair loss, thinning, orlow density of hair were excluded using relevantindividualized investigations if suspected. All casesdiagnosed as scarring hair loss were also excluded.

For the hair pull test, a bundle of 50 to 60 hairs wasgrasped between the thumb and index finger near thescalp. Firm, but not forcible, slow, steady traction wasapplied beginning from the scalp surface to the hairends with the fingers sliding along the hair shaft. Thenumber of removed hairs was then counted and theywere examined under a light microscope. More than10% of grasped hairs pulled away from the scalp isconsidered a positive pull test. The same procedurewas repeated in the frontal, right and left parietal, andoccipital areas.

A trichogram was performed 3 days after the lasthair wash. At least 50 hairs were grasped with a pair of

Address correspondence to Vivek Dey, M.D., Department ofDermatology, Venereology and Leprosy, People’s College ofMedical Sciences & Research Centre, Bhopal, MP, India,Pin-462037, or e-mail: drvivekdey@yahoo.co.in.

DOI: 10.1111/pde.12208

© 2013 Wiley Periodicals, Inc. 579

Pediatric Dermatology Vol. 30 No. 5 579–583, 2013

artery forceps with micropore tape over the tipsplaced approximately 0.5 cm above the scalp androtated to ensure a firm grip. Hairs were removed witha firm, quick, forceful pull along the direction of hairgrowth and examined under a light microscope. Hairsamples were obtained from the frontal and occipitalregions in cases of diffuse thinning of hair and fromthe margin of clinically involved areas in cases withpatchy hair loss. Diagnosis of LAHS was made on thebasis of the revised diagnostic criteria for LAHS byTosti et al (the presence of 70% or more loose anagenhairs on trichogram) (2).

RESULTS

The results are summarized in Table 1. During thespecified period, 526 children were examined fornonscarring hair loss, thinning, or low density of hair.Eight were diagnosed with LAHS on the basis ofclinical and microscopic findings after careful exclu-sion of other causes. The male:female ratio in thisgroup was 1:1. The age of diagnosed children was 2 to10 years (mean 4.6 years). The main complaints weresparse hair, slow growth, and easily and painlesslypluckable hair in most of the patients. Two patients(patients 3 and 5) complained of slow growth and hairshedding but had near-normal hair density. They gavea history of improvement with age. Patients 1 and 4also complained of unmanageable, unruly hair. Onexamination the low density of hair was diffuse (exceptpatients 3 and 5), with some accentuation on the vertexor parietal or occipital areas (Fig. 1). In two patients(patients 1 and 4), occipital hairs were unmanageableand unruly. In all cases disease was confined to scalphair.None of our patients had clinically visible areas ofcomplete hair loss. Patient 4 also had atopic dermatitisand patient 8 had generalized xerosis. Nails, teeth, andeyes were normal in all cases. The general health of allof the children was good, diet was normal, and therewas no history of habitual hair pulling in any case.

In the hair pull test, 5 to 18 hairs (mean 10.4) cameout easily and painlessly from each site in all cases.The hair pluck trichogram was also almost painless inall patients. On trichogram, there was a strikingpredominance of anagen hair in all cases (75%–100%), absent inner and outer root sheaths, ruffling ofthe cuticle on the proximal hair shaft, and misshapenhair bulbs (Fig. 2).

DISCUSSION

Zaun first described loose anagen hair in 1984 as a“syndrome of loosely attached hair in childhood” at T

ABLE

1.Subject

Characteristics

Characteristic

Patient1

Patient2

Patient3

Patient4

Patient5

Patient6

Patient7

Patient8

Sex

Fem

ale

Male

Male

Male

Fem

ale

Fem

ale

Male

Fem

ale

Age

22.5

85

10

33

4Durationofhairfall

6mos

3mos

5yrs

(lessseverenow)

2yrs

7yrs

(less

severenow)

1yr

6mos

2yrs

Consanguinityin

parents

No

No

No

No

No

No

No

No

Familialoccurrence

–Yes

Yes

Yes

Yes

––

–Relationship

toother

cases

––

Brother

of

patient2

–Sisterof

patient4

––

–

Unruly

hair

Present

––

Present

––

––

Loose

anagen

type

AA

CA

CA

AA

Areaswherethinning

wasaccentuated

Vertex,occipital

Both

parietal

No

Vertex,both

parietal

No

Parietal,temporal,

occipital

No

No

Hairsremoved

onhair

pulltest,n*

6+10+14

+15+10=55

(mean=11)

10+16+14

+11+8=59

(mean=11.8)

5+10+8

+7+13=43

(mean=8.6)

11+15+12

+10+10=58

(mean=11.6)

10+8+11

+6+5=40

(mean=8)

7+12+11

+10+8=48

(mean=9.6)

11+8+10

+15+12=56

(mean=11.2)

7+18+12

+12+10=59

(mean=11.8)

Loose

anagen

inhairpluck

trichogram,%

100

95

80

85

75

100

90

90

Associatedectodermal

disorders

None

None

None

None

None

None

–Xerosis

Associateddermatoses

––

–Atopic

dermatitis

––

––

*Hairsfrom

fiveareasare

taken

inthehairpulltest:frontal,rightparietal,leftparietal,vertex,andoccipital.

580 Pediatric Dermatology Vol. 30 No. 5 September/October 2013

the First Congress of the European Society forPediatric Dermatology (Munster, Germany, October4–7, 1984). N€odl et al then reported it in the Germanliterature in 1986. Zaun first reported it in the English-language literature in 1987 under the name of “syn-drome of loosely attached hair in childhood” (3). Afew years later, Hamm and Traupe (4) and Price andGummer (5) coined the term LAHS and first pub-lished it in the American literature in 1989. Thecommon necessary finding in all these reports waspainless extraction of anagen hair lacking inner andouter root sheaths.

Since then, many researchers have described theoccurrence of LAHS in white children, especially girlswith blonde hair. To our knowledge, our study is the

first report of LAHS in dark-skinned children fromIndia. The first study of LAHS on dark-skinnedindividuals was by Abdel-Raouf et al (1). In initialreports, the incidence of LAHS was estimated to be 2to 2.5 patients per million per year, with a sex ratio of6 boys to 37 girls (6). In our study, LAHS wasdiagnosed in 1.5% of all cases of nonscarring hair lossin children younger than 12 years old attending ourclinic. We found an equal sex ratio, further supportingautosomal dominant inheritance proposed by Badenet al (7). We also found LAHS in two families in ourstudy. Pham et al (8) proposed that LAHS may beunderdiagnosed in boys simply because of hairstyledifferences between boys and girls.

LAHS results from premature keratinization of theinner root sheath that produces impaired adhesionbetween the cuticle of the inner root sheath and thecuticle of the hair shaft (2). The most conspicuousstructural changes found in the inner root sheathcomplex of the anagen follicle is keratinization andirregular swelling. The major pathologic changes ofintercellular edema in the prekeratinized Huxley cellzone and dyskeratosis of Henle cells and cuticle cellsof the inner root sheath can be seen using electronmicroscopy (9). Mutations in the gene encoding forkeratin K6HF and K6IRS have been found (2,10).

Clinical presentation is heterogeneous. Three phe-notypes have been identified: type A, with sparse hairthat does not grow long; type B, characterized bydiffuse or patchy, unruly hair; and type C,characterized by normal-appearing hair with exces-sive shedding of loose anagen hairs. In ourstudy, patients 3 and 5 were type C LAHS, and theothers were type A, with unruly hair in patients 1 and

A B

Figure 1. (A) Patient with LAHS showing sparse hair that is accentuated in the occipital area (patient 1). (B) Patient 2shows sparse thin hair with accentuation in both parietal areas.

Figure 2. Characteristic trichogram of loose anagen hairshowing ruffled proximal cuticle (arrow 1), misshapen hairbulb, shaft bulb angulation (arrow 2), and absence of theinner and outer root sheath.

Dey et al: Loose Anagen Hair Syndrome 581

4. Type A and B phenotypes almost exclusively affectchildren, whereas type C predominantly affects adults.There is a tendency for the clinical presentation ofpatients with LAHS types A and B to evolve intoLAHS type C with age (11). Involvement of body hairand eyebrows has rarely been reported (12) and all ofour cases involved the scalp only.

Diagnosis relies on the number and percentage ofhairs in the pull test and loose anagen hairs in thetrichogram. In the case of LAHS, the pull test revealsmore than 3 to 10 hairs easily and painlessly plucked(13), and the trichogram shows 70% to 100% looseanagen hairs. A striking predominance of anagenhair, the absence of telogen hair, absent inner andouter root sheaths, ruffling of the cuticle on theproximal hair shaft (floppy sock appearance), andmisshapen hair bulbs that may appear long andtapered, twisted, or positioned at an acute angle to thelong axis of the hair shaft (mousetail-like) (1) arecharacteristic findings in the trichogram. Slight flat-tening and longitudinal grooving has been noted onelectron microscopy (4).

Tosti et al (14) proposed diagnostic criteria forLAHS that include positive pull test results withpainless extraction of at least 10 loose anagen hairsand the presence of more than 80% loose anagen hairson trichogram, but these criteria were too strict topermit individuals who are mildly affected to bediagnosed. Therefore they revised these criteria andsuggested that the diagnosis of LAHS should be madeif the trichogram shows at least 70% loose anagenhairs (2). Cantatore-Francis and Orlow (15) proposedthat LAHS should be diagnosed only when there aremore than 50% loose anagen hairs on the trichogram.

There is no obvious relationship between theclinical severity of the condition and the number ofloose anagen hairs that can be obtained on the pulltest. Even in patients with widespread LAHS, notevery anagen hair on the scalp is loose. Therefore, ifthe diagnosis of LAHS is suspected on clinicalgrounds, a negative hair pull test does not excludethe diagnosis, and a hair pluck trichogram should beperformed (10).

In most cases LAHS is not associated with othermedical conditions. One of our cases had associatedatopic dermatitis and one had generalized xerosis.Two children with LAHS associated with atopicdermatitis were been reported in 2009 (15), but thisassociation may be coincidental given the common-ality of atopic dermatitis in childhood. Other associ-ated disorders reported in the literature are coloboma(16,17), Noonan syndrome (18,19), hypohidroticectodermal dysplasia (20), ectrodactyly–ectodermal

dysplasia–clefting syndrome, trichorhinophalangealsyndrome, nail patella syndrome, neurofibromatosis,FG syndrome, woolly hair (2,15,21), alopecia areata(22), and AIDS (23).

CONCLUSION

Ourfindings confirm thatLAHSexists in dark-skinnedchildren, and the equal male:female ratio suggestsunderdiagnosis in boys. A high degree of suspicion isrequired for the diagnosis in young children withnonscarring hair loss and a history of sparse, thin hair.Careful history, clinical findings, and a hair plucktrichogram should easily exclude similar hair disorderssuch as alopecia areata (22), trichotillomania (24),telogen effluvium, anagen effluvium, and short anagensyndrome (25). Proper counselling and reassurance topatients and parents is important. Observation andgentle care of hair is the treatment of choice becauseLAHS usually improves spontaneously with age.

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