hairlossin children - bmj · other causes of scarring alopecia include ... irritation is not a...

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Archives ofDisease in Childhood 1993; 68: 702-706 PERSONAL PRACTICE Hair loss in children Julian Verbov There are many causes of hair loss in children and I shall mention some of these (table), but I will deal in more detail with alopecia areata and with children who pull their hair. Normal hair growth in children has been reviewed recently. ' Aplasia cutis and other scarring alopecias Congenital absence of skin (aplasia cutis) presents on the scalp as one or more non- inflammatory well defined oval or circular ulcers, crusted areas (fig 1) or as scars. Lesions usually occur over the vertex in or adjacent to the midline and may involve skin only or occasion- ally may extend deeply to bone and dura. Complications include secondary infection, bleeding, and meningitis with deeper lesions. Occasionally other developmental defects are present and there may be a family history of aplasia cutis.2 PROGNOSIS AND MANAGEMENT Most lesions are superficial and heal over a period of many weeks, leaving an area of scarring alopecia and this is how they often present. Larger defects may require excision and use of tissue expanders before closing the deficit. Before plastic surgery use of protective helmets and rubber skull caps will protect the aplastic area. Other causes of scarring alopecia include physical injuries such as burns or uncommonly after prolonged localised scalp pressure as at Causes of hair loss in children Aplasia cutis/other scarring alopecias Sebaceous naevus Hereditary Telogen effluvium Chemical Endocrine Nutritional Ringworm Alopecia areata Trauma: Traction Loose anagen Shaft defects Pulled hair surgical operation, severe bacterial, viral, or fungal infections and uncommon conditions such as chronic folliculitis, sarcoidosis, or fronto- parietal morphoea. Sebaceous naevus This is an uncommon, usually small, congenital lesion containing both epidermal and dermal elements. Most common over the scalp and usually single, it appears as a smooth slightly raised hairless waxy plaque, yellow orange in colour and linear or slightly oval in shape. It becomes thickened and more raised during late childhood and adolescence (fig 2). Benign or malignant transformation, particularly basal cell carcinoma, is not uncommon in these lesions and usually occurs from the fourth decade: excision is thus advised in adolescence or early adult life. Hereditary hair loss When occurring as an isolated finding, hair loss Royal Liverpool Children's NHS Trust, Myrtle Street Children's Hospital, Myrtle Street, Liverpool L7 7DG Correspondence to: Dr Verbov. Figure I Aplasia cutis. Figure shows a 3 week old infant with crusted area. Local infection responded to topical applications. The boy also had congenital heart disease. Ten years on he is fine after scalp and cardiac surgery. He has a cousin with minor aplasta cutis as an isolated finding. Figure 2 Sebaceous naevus. Figure shows a raised hairless scalp naevus in a 17 year old male. 702 on January 11, 2021 by guest. Protected by copyright. http://adc.bmj.com/ Arch Dis Child: first published as 10.1136/adc.68.5.702 on 1 May 1993. Downloaded from

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Page 1: Hairlossin children - BMJ · Other causes of scarring alopecia include ... irritation is not a prerequisite for hair regrowth withdithranol. ... Children who pull their hair are relatively

Archives ofDisease in Childhood 1993; 68: 702-706

PERSONAL PRACTICE

Hair loss in children

Julian Verbov

There are many causes of hair loss in childrenand I shall mention some of these (table), but Iwill deal in more detail with alopecia areata andwith children who pull their hair.Normal hair growth in children has been

reviewed recently. '

Aplasia cutis and other scarring alopeciasCongenital absence of skin (aplasia cutis)presents on the scalp as one or more non-inflammatory well defined oval or circular ulcers,crusted areas (fig 1) or as scars. Lesions usuallyoccur over the vertex in or adjacent to themidline and may involve skin only or occasion-ally may extend deeply to bone and dura.Complications include secondary infection,bleeding, and meningitis with deeper lesions.Occasionally other developmental defects arepresent and there may be a family history ofaplasia cutis.2

PROGNOSIS AND MANAGEMENTMost lesions are superficial and heal over aperiod ofmany weeks, leaving an area of scarringalopecia and this is how they often present.Larger defects may require excision and use oftissue expanders before closing the deficit.Before plastic surgery use of protective helmetsand rubber skull caps will protect the aplasticarea.

Other causes of scarring alopecia includephysical injuries such as burns or uncommonlyafter prolonged localised scalp pressure as at

Causes ofhair loss in children

Aplasia cutis/other scarring alopeciasSebaceous naevusHereditaryTelogen effluviumChemicalEndocrineNutritionalRingwormAlopecia areataTrauma:

TractionLoose anagenShaft defectsPulled hair

surgical operation, severe bacterial, viral, orfungal infections and uncommon conditions suchas chronic folliculitis, sarcoidosis, or fronto-parietal morphoea.

Sebaceous naevusThis is an uncommon, usually small, congenitallesion containing both epidermal and dermalelements. Most common over the scalp andusually single, it appears as a smooth slightlyraised hairless waxy plaque, yellow orange incolour and linear or slightly oval in shape. Itbecomes thickened and more raised during latechildhood and adolescence (fig 2). Benign ormalignant transformation, particularly basal cellcarcinoma, is not uncommon in these lesions andusually occurs from the fourth decade: excisionis thus advised in adolescence or early adult life.

Hereditary hair lossWhen occurring as an isolated finding, hair loss

Royal LiverpoolChildren's NHS Trust,Myrtle Street Children'sHospital, Myrtle Street,Liverpool L7 7DGCorrespondence to:Dr Verbov.

Figure I Aplasia cutis. Figure shows a 3 week old infantwith crusted area. Local infection responded to topicalapplications. The boy also had congenital heart disease. Tenyears on he is fine after scalp and cardiac surgery. He has acousin with minor aplasta cutis as an isolated finding.

Figure 2 Sebaceous naevus. Figure shows a raised hairlessscalp naevus in a 17 year old male.

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Hair loss in children

Figure 3 Hypohidrotic ectodermal dysplasia. Figure showsa 14 month old boy with sparsefair hair and characteristicprofile.

is usually an autosomal dominant trait andeyebrows and eyelashes may be affected as wellas the scalp and loss is usually permanent.However, sparse hair is most commonly just onecomponent in many genodermatoses such as

hypohidrotic (fig 3) (X linked recessive) andhidrotic (autosomal dominant) ectodermaldysplasia, Rothmund-Thomson syndrome(autosomal recessive), focal dermal hypoplasia(X linked dominant - affected individuals mayalso get aplasia cutis), and acrodermatitisenteropathica (autosomal recessive).

Telogen effluviumAny severe physical (for example febrile illness,surgery) or mental stress (for example depres-sion) may induce hair loss. Typically, diffusehair loss occurs 3-4 months after the stressbecause of premature conversion of growing(anagen) hairs to the resting phase (telogen): losscontinues for a few months but regrowth follows.The temporary diffuse loss of hair in infants

during the first few months of life and maternalpostpartum hair loss are other examples oftelogen effluvium but both head movement andpressure also contribute to the common occipitalhair loss in infants.

Chemical hair lossToxic hair loss is seen with antimetabolites,alkylating agents, and mitotic inhibitors all ofwhich inhibit synthesis of hair in growing(anagen) follicles and hair is lost (anageneffluvium). Regrowth usually recurs when drugadministration is discontinued: irradiation can

similarly cause temporary or permanent scalphair fall.

Hypervitaminosis A, synthetic retinoids, anti-

thyroid drugs, anticoagulants, and sodiumvalproate may cause hair loss and many otherdrugs have been reported as causing alopecia butincriminating evidence and mode of causationare not always clear.

Endocrine causesHair loss may be seen in hypopituitarism, hypo-thyroidism and hyperthyroidism,3 hypopara-thyroidism, and in poorly controlled diabetesmellitus.

Nutritional deficiencyHair loss may occur with deficiencies of iron,biotin, zinc, and essential fatty acids and, ofcourse, in marasmus. Diffuse alopecia is one ofthe features of anorexia nervosa.4

Scalp ringwormRingworm affecting the scalp (tinea capitis) isprimarily a disease of children.5 Organismsinvade the hair shaft and stratum corneum of theepidermis.

DIAGNOSISInfection is acquired from other humans (that isanthropophilic) or animals (that is zoophilic) andclinical appearances depend on infecting speciesand host inflammatory response. The mostmarked presentation with swollen pustular areasis known as kerion and is usually caused by azoophilic species. Infection from cats and dogs(due to Microsporum canis) is characterised byhair loss and broken off hairs with a varyingdegree of scalp erythema and scaling. Hairsinfected with Microsporum audouinii (an anthro-pophilic fungus) or M canis fluoresce greenunder a Wood's light and this is a useful massscreening procedure. M canis predominates as acause of tinea capitis in the UK, and Trichophytontonsurans dominates in the USA mainly affectingthe black population. Trichophyton violaceum isthe most common cause in India, Kenya, andparts of North Africa and Trichophytonsoudanense (fig 4) is important in Central andWest Africa. Immigrants can bring such speciesinto Britain but even when born in Britain suchchildren may be infected by fungi endemic intheir countries of origin.A diagnosis of ringworm can be confirmed by

observing fungal elements in microscopic prep-arations softened with potassium hydroxide, butculture is required to identify the particularfungus concerned.

MANAGEMENTWith anthropophilic infections children shouldbe kept away from school for a short period ifpractical, until they are non-infective and aftercontacts have been screened, because of the riskof spreading infection to other children. Intreating scalp ringworm oral griseofulvin (10 mg/kg body weight/day) is prescribed for 4-6 weeksin addition to a topical imidazole antifungal and amedicated shampoo.

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Verbov

Figure 4 Scalp ringworm (T soudanense). Patchy scalp hairfall appeared in these 6year oldNigerian twin girlsfour months after amving in Liverpool. Affected areas showed mildinflammation and scaling.

Alopecia areata6Alopecia areata has been recognised since anti-quity and is recorded in the Papyrus Ebers withsuggested remedies.7 It is the most common formofhair loss in children but an onset before 2 yearsis unusual. No specific cause has been foundbut it is likely that an immune mechanism isinvolved. A small percentage of affected indi-viduals show an increased frequency of organspecific autoantibodies directed against varioustissues. Up to one third of patients have a familyhistory of the condition (fig 5). Stress has apossible precipitating role in some cases.The initial event in alopecia areata is prema-

ture entry of anagen follicles into telogen,although some follicles survive for a time in adystrophic anagen state.'

PFF IFigure 5 Alopecia areata. Patch ofhairfall occurred at thesame time in father (aged 24) and son (aged 4).

DIAGNOSISOne or more sharply defined oval or roundpatches of complete hair loss produce an eggshell appearance over the scalp, although anyhair bearing area may be affected. Early patchesmay show an irregular outline. The disorder ischaracteristically asymptomatic. In the activephase, pathognomonic club hairs, with brokenoff tips, so called exclamation mark hairs, may beseen, particularly at the margin of the area of hairloss. Regrowing hair initially tends to be fine andunpigmented.

MANAGEMENTI explain the condition in simple terms to parentsand older children emphasising the good prog-nosis with scanty patches and being more waryabout widespread or recurrent loss.

Because of the likelihood of spontaneousregrowth, I commonly prescribe a shampoo, anda mild cream such as sulphur 2%, salicylic acid2% in aqueous cream BP. For more extensiveloss I often prescribe dithranol, usually in theform of a cream; initially 0-1% is applied for 10-30 minutes at night, and then washed off withsoap and water or shampoo; the cream may stainthe skin and concentration should not beincreased if irritation is at all marked; however,irritation is not a prerequisite for hair regrowthwith dithranol. I have no personal experience ofthe use of topical immunotherapy9 and I do notnormally prescribe corticosteroids in any form. Irecommend a wig for alopecia totalis in thepreschool child but the older child may be moreembarrassed wearing one than not doing so. Awig is indicated for extensive alopecia areata inDown's syndrome, a disorder associated with anincreased frequency of the condition.

Figure 6 Trichotillomnania. Girl aged 9 vears showingbizarre distribution ofhair loss.

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Hairloss in children

PROGNOSISThe prognosis is generally good when there arefew patches of hair loss, with likely regrowth in6-12 months. However, the condition mayrecur. The more extensive the loss, the moreguarded should be the prognosis. Occipitalpatches tend to regrow very slowly. When associ-ated with atopy prognosis also tends to be poor.Nail changes including pitting and distortion ofthe nail plate, sometimes occur, particularlywith extensive alopecia. Rarely, all scalp hair(alopecia totalis) or all body hair (alopeciauniversalis) may be lost and this tendency seemsto be greater in children than adults.

Traumatic hair lossTraction alopecia can be caused by accidentaltrauma such as various ethnic hair styles, ponytails and other trendy styles, tight rollers, andhot combing. Hair loss may occur in atopicdermatitis due to persistent rubbing and in scalppsoriasis where loosened hair may be lost.

Loose anagen syndrome is a recently describedentity'" in which the hair is not fragile but iseasily and painlessly plucked: it seems to occur

more commonly in fair haired young childrenand improves with time. Some familial cases

have been reported."Structural defects ofthe hair shaft (visible micro-

scopically) may be associated with increasedfragility and may present spontaneously as hairloss or as loss after mild trauma. Such defectsinclude the most common one trichorrhexisnodosa (nodes easily fracturing with trauma) andthe autosomal dominant pili torti (twisting of hairthat appears spangled). These defects may occuras isolated findings or in conditions such as thesex linked recessive Menkes' (kinky hair) syn-drome and the autosomal recessive Netherton'ssyndrome, although a bamboo-like hair defect(trichorrhexis invaginata) is more characteristic ofNetherton's syndrome.'2 Trichorrhexis nodosamay occur in the dominant hereditary woollyhair. Monilethrix (beaded hair) is another shaftdefect, inherited as a dominant trait.'3 Trichothio-dystrophy describes brittle hair with a lowsulphur content'"; it is inherited as an autosomalrecessive trait.

Scalp hair fall may also be inflicted by others.

Children who puli their hairChildren who pull their hair are relativelycommon but the problem receives little or noattention in standard paediatric texts. Affectedchildren and even their parents may sometimesbe unaware that the hair loss is self inflicted. It isseven times more common in children than inadults.'5 Although most affected individuals aregirls, under the age of6 years it'is more commonin boys.The term 'trichotillomania' was coined by

Hallopeau"6 to emphasise the 'pulling', althoughBesnier preferred 'trichomania'. One shouldnote that the term mania is not being used in itswell defined modern sense. The Diagnostic andStatistical Manual of Mental Disorders of theAmerican Psychiatric Association defines tricho-tillomania as an irresistible urge to pull the hair

and a sense of relief after the hair has beenplucked. 7

AETIOLOGYHair pulling may occur as an isolated habit, outof the blue, and without obvious explanation.Minor pulling and hair loss is a habit commonlyassociated with boredom. In children with hairloss sufficiently marked to be referred to ahospital dermatology clinic hair pulling isusually a sign of chronic social deprivation andparticularly emotional deprivation in thematernal relationship; in a few of these childrenthere may be progress to obsessive-compulsivedisorder. Occasionally, neuroses such as anxietyand obsessive-compulsive disorder may beprimary. Rarely, depression is the underlyingcause.

DIAGNOSISThe younger child often admits to pulling thehair but the more severely affected older childusually denies touching the hair. I do not usuallyask a patient whether their loss is self inflicted atleast not until I've seen him/her a few times andthere is good rapport. Sometimes their com-ments can be revealing: one older girl whodenied pulling said to me that 'as my hairregrows it feels like it is being pulled out'.

Hair is lost most frequently from a fronto-parietal region but the loss can affect elsewhere.In the affected area twisted hairs and brokenhairs of varying length will be visible. In moresevere cases plucking may be extensive so thatonly a margin of hair remains. Rarely hair awayfrom the scalp may be plucked. Although clinicaldiagnosis is usually straightforward with theaffected area appearing bizarre and ill defined,differential diagnosis includes ringworm wherethe bald areas are usually scaly, and alopeciaareata. In contrast to alopecia areata, histo-pathology in trichotillomania reveals neitherinflammation of the hair bulb nor atrophicanagen hairs.'8

It is quite common for hair loss due to pullingto be preceded by an episode of alopecia areata;perhaps the attention given activates an inherentsusceptibility to pull in these children. The verycommon nail biting (onychophagia) and nailpulling (onychotillomania) may coexist with hairpulling. Bulimia nervosa has been reported tofollow trichotillomania. 9 Pulled hairs may some-times be swallowed (trichophagy) and can resultin intestinal obstruction caused by a hairball(trichobezoar).

Case historiesCASE 1A 9 year old girl presented with a one monthhistory of increasing scalp hair fall. On examina-tion, there was marked scalp hair thinning with amargin of good hair remaining over sides andposterior scalp; the thinned area showeddifferent lengths of stubble (fig 6). Her motherand a schoolteacher had noticed the child twist-ing her hair. The mother tended to push her ather studies and to direct her leisure activities, for

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example no dolls. Her father was a more easygoing individual and she had two brighteryounger brothers. I arranged for the child to seeboth a paediatrician and a child psychiatrist inaddition to myself. Full regrowth of hair occur-red within nine months. Her mother neveraccepted that the hair loss was solely selfinflicted.

CASE 2A 5-5 year old boy came to clinic with a history ofa patch of alopecia areata the previous year thathad regrown within three months. One monthafter regrowth bifrontal hair loss occurred. Themother had noticed him pulling his hair and headmitted it to me also on careful questioning,and the scalp appearance was bizarre. There wasa history of nail biting before the alopecia areata.It became clear that hair pulling usually occurredat school, regrowing during the holidays: he hadlearning difficulties at school and teacher prob-lems. He was prescribed a bland cream to applyto the scalp and the hair regrew. The hairreturned to normal over a 10 month period.However, once the hair had regrown he beganbiting his nails again and I told him to apply thescalp cream to the nails rather than bite them.The nail biting ceased rapidly when he becamehappier in school.

MANAGEMENT AND PROGNOSISManagement depends on detecting the reasonfor hair pulling. If an isolated habit, behaviouralmanagement should help. Ifdue to chronic socialdeprivation this has to be sorted out andimprovement in the quality of child care at homeand in the community must be a priority. If ananxiety state is present, family or individualpsychotherapy may be helpful. Clomipramine (aserotonin reuptake blocking drug) may have aplace in the severely affected older child withobsessive-compulsive disorder but advice should

be sought from a child psychiatrist. Somesuccess has been claimed in the probablyanalogous conditions ofcompulsive feather pick-ing in birds20 and compulsive paw licking indogs.2'

In practice, prognosis in the young child isgenerally good but is more guarded in theadolescent female with marked hair loss.

1 Barth JH. Normal hair growth in children. Pediatr Dermatol1987; 4: 173-84.

2 Sybert VP. Aplasia cutis congenita: a report of 12 new familiesand review of the literature. Pediatr Dermaeol 1985; 3: 1-14.

3 Heymann WR. Cutaneous manifestations of thyroid disease. JAm Acad Dermatol 1992; 26: 885-902.

4 Miller SJ. Nutritional deficiency and the skin. J Am AcadDermatol 1989; 21: 1-30.

5 Clayton YM. Scalp ringworm (tinea capitis). In: Verbov JL,ed. Superficial fungal infections. Lancaster: MTP Press Ltd,1986: 1-19.

6 Thiers BH, Bergfeld WF, Fiedler-Weiss VC, et al. Alopeciaareata symposium. PediatrDermatol 1981- 4: 136-58.

7 Ebbell B. The papyrus ebers: the greatest Egyptian medicaldocument. Copenhagen: Levin and Munksgaard, 1937.

8 Messenger AG, Slater DN, Bleehen SS. Alopecia areata:alterations in the hair growth cycle and correlation with thefollicular pathology. BrJ Dermatol 1986; 114: 337-47.

9 MacDonald Hull SP, Pepall L, Cunliffe WJ. Alopecia areata inchildren: response to treatment with diphencyprone. BrJDermatol 1991; 125: 164-8.

10 Zaun H. Differential diagnosis of alopecia in children. In:Happle R, Grosshans E, eds. Pediatric dermatology. Berlin:Springer, 1987: 157-66.

11 Baden HP, Kvedar JC, Magro CM. Loose anagen hair as acause ofhereditary hair loss in children. Arch Dermatol 1992;128: 1349-53.

12 Krafchik B. Netherton syndrome. Pediatr Dermatol 1992; 9:157-60.

13 Ito M, Hashimoto K, Katsuumi K, Sato Y. Pathogenesis ofmonilethrix: computer stereography and electron micro-scopy.J Invest Dermatol 1990; 95: 186-94.

14 Itin PH, Pittelkow MR. Trichothiodystrophy: review ofsulfur-deficient brittle hair syndromes and association withthe ectodermal dysplasias. J Am Acad Dermatol 1990; 22:705-17.

15 Mehregan AH. Trichotillomania: a clinopathologic study.Arch Dermatol 1970; 102: 129-33.

16 Hallopeau FH. Alopecie par grattage (trichomanie outrichotsllomanie). Annales Dermatologie et de Syphiligraphie1889; 10: 440-1.

17 Swedo SE, Rapoport JL. Annotation: trichotillomania.J Child Psychol Psychiatry 1991; 32: 401-9.

18 Muller SA. Trichotillomania: a histopathologic study in sixty-six patients.J AmAcad Dermatol 1990; 23: 56-62.

19 George MS, Brewerton TD, Cochrane C. Trichotillomania(hair pulling). N EnglJ Med 1990; 322: 470-1.

20 Grindlinger HM. Compulsive feather picking in birds. ArchGen Psychiatry 1991; 48: 857.

21 Rapoport JL. Treatment of behavioural disorders in animals.AmJ7Psychiatry 1990; 147: 1249.

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