Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405)

Hair Loss in Children

Rubina Alves · Ramon Grimalt Department of , Universitat Internacional de Catalunya, Barcelona, Spain

Abstract Introduction diseases represent frequent complaints in dermatol- ogy clinics, and they can be caused by a number of condi- in children occurs in a wide range of tions reflected by specific diagnoses. Hair loss is not un- conditions that may be congenital or acquired. A common in the pediatric group, but its patterns in this congenital abnormality may be an isolated find- group are different from those seen in adults. Addition- ing in a healthy patient or may occur as a feature ally, in children, these disorders can have psychological of a multisystem syndrome. Recognition of a hair effects that can interfere with growth and development. disorder may enable the diagnosis of a particular Hair is easily accessible for examination, and dermatolo- syndrome. The clinical presentations of pediatric gists are in the enviable situation of being able to study hair disorders range from subtle to disfiguring. many disorders using simple diagnostic techniques. To Alopecia is not uncommon in the pediatric popu- fully understand hair loss during childhood, a basic com- lation but has patterns that are different from prehension of normal hair growth is necessary. Knowl- those seen in adults. The occurrence of these edge of the normal range and variation observed in the problems during childhood can cause psycholog- hair of children further enhances its assessment. This ical and emotional to both children and chapter has been written in an attempt to facilitate the their parents. A good knowledge of the normal diagnostic process during daily practice by helping to dis- hair cycle, embryology and clinical features is tinguish between acquired and congenital hair diseases. necessary. It can sometimes be difficult to differentiate between ab- normality and normality in neonatal hair aspects. Man- agement of hair disorders can be quite a daunting task Embryology and Normal Hair Development for the attending physician and mandates a holistic ap- proach to the patient. Some hair disturbances have no Hair follicles are derived from an interaction be- effective treatment, and for others, no single treatment is tween the embryological ectoderm and meso- 100% successful. If no effective treatment for a hair loss derm, which begins at 9 weeks of gestation. Pri- disease exists, a cosmetic approach is important. mary follicles first develop on the , up- © 2015 S. Karger AG, Basel per lip and chin. Then, hair follicles develop over Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM the in a frontal to occipital direction and tailed history is essential for an accurate diagno- progress over the body in a cephalocaudal direc- sis. The key points in a patient’s history are the tion [1]. By 18–20 weeks of gestation, the entire following: age of onset (congenital or acquired); initial population of follicles has formed, includ- onset of hair loss (sudden or insidious); extent of ing those on the scalp [2]. Each follicle is capable alopecia (localized or diffuse); physical and men- of producing three different types of hair as fol- tal development (underlying syndrome?); past lows: , vellus and terminal. Lanugo hair is medical history (, , nonmedullated, fine, soft, and usually nonpig- or ); psychiatric disorders; and family mented, and it can be found on the bodies of history of alopecia [3, 5]. Similar to the history newborns. This type of hair is shed by 3–4 months evaluation, an exhaustive after birth. is short, fine, light-col- should be performed to assess the following ored, barely noticeable, and covers almost the components: type of alopecia (localized or gen- whole body. During , the hor- eralized/scarring or nonscarring); hypotrichosis mone causes most of vellus hair to turn into ter- or alopecia; hair shaft anomalies; hair quality; minal hair. is larger, thicker and and hair color. A thorough scalp examination strongly pigmented, and it is found on the scalp, is also important to evaluate the existence of eyebrows, axillary and pubic areas, chest and face ­, edema, pustules, scaling, atrophy or [1]. scarring. Human hair grows in a continuous cyclic pat- The presence of short stature, abnormal bone tern known as the hair cycle. The hair cycle pres- development, defective hearing, dysmorphic fea- ents with different phases as follows: the anagen tures, impaired vision or other physical findings or growth phase (85–90% of ), the catagen or could indicate an underlying metabolic or auto- regression phase (<1% of hairs), the telogen or immune disease. resting phase (4–15% of hairs) and, finally, the Usually, the diagnosis of the most common shedding exogen phase. At birth, about 5 million forms of hair loss can be made only by clinical and hair follicles cover the human body, and approxi- physical examinations. A hair pull test distin- mately 100,000 are scalp hairs [2]. Newborn hairs guishes between loss from follicles and loss due to are all anagen, and during childhood, there is a hair shaft fragility. To perform this test, around gradual transition of scalp hairs from vellus to 50–60 hairs are grasped between the index finger terminal hairs [3]. and thumb and then lifted with gentle traction. The pull test is considered positive if more than 10% of hairs are released [5]. False positives can Diagnosis occur if the test is performed on a day in which hair has been washed. A newborn can have the following three presen- To confirm breakage of the hair shaft, a tug test tations of hair (normal variants): a full head of should be performed. The tug test consists of the hair, or little or no hair. The beginning of abnor- grasping of a hair between the finger and thumb mal hair growth sometimes occurs during infan- near its exit from the scalp and the pulling of the cy; thus, it is difficult to predict which newborn distal part [6]. If the hair is fragile, a fracture will will have a hair pathology. Knowledge of a pa- occur in the shaft. Trichoscopy, a noninvasive tient’s personal and family history, a thorough method, has emerged as a valuable tool in the dif- clinical examination, as well as general and spe- ferential diagnosis of most hair and scalp diseases. cific diagnostic procedures are important for a It is also important to evaluate the therapeutic re- correct diagnosis and early treatment [4]. A de- sponse of hair loss [7].

56 Alves · Grimalt

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM fect the scalp or any hair-bearing area on the body. This disorder is associated with in- Alopecia areata (AA) is a nonscarring, autoim- volvement, including nail pitting, trachyonychia, mune and inflammatory pattern of alopecia that brittle nails, and [16– occurs in both children and adults. It is a com- 18]. mon disorder, affecting different ethnicities with Trichoscopy examination reveals the pres- equal incidences between genders. The preva- ence of short ‘exclamation mark’ hairs at the pe- lence of AA is approximately 0.2% in the general riphery of the (pathognomonic of AA) and population, and its lifetime risk is estimated to be ‘yellow dots’ in a follicular distribution. The hair approximately 1.7% [8]. Although AA has been pull test is typically positive in active AA with the considered rare in young infants, recent studies presence of telogen club hairs and dystrophic have suggested that this disorder could occur in anagen hairs [5, 19]. A scalp biopsy is usually un- 1–2% of patients less than 2 years of age [9]. In necessary to establish the diagnosis of AA, except children younger than 16 years of age, AA has in the case of diffuse shedding. The hallmark his- been reported to occur in 21–24% of patients [9, tological finding is a dense infiltrate 10]. comprising mainly T cells around the anagen The predisposition to AA is genetically deter- hair bulb matrix and the dermal papillae. The mined, with 5–25% of patients possessing a strong main of AA in children in- family history [10, 11]. There is also an increased cludes , (TTM), frequency of AA in individuals with Down syn- transient neonatal hair loss (TNHL) and congen- drome [12–15]. AA may be seen in association ital triangular alopecia. with autoimmune disorders, such as atopic der- Tinea capitis is a common cause of patchy matitis, allergic rhinitis, , and au- hair loss in infants, although individuals of all toimmune thyroid disease [13]. ages are occasionally affected. This condition in- There are several clinical presentations of AA, volves the invasion of scalp hairs by dermato- which are usually classified according to the hair phyte fungi, including Trichophyton and Micros- loss pattern or extent. The classic presentations porum species. The clinical picture of this disease are as follows: patchy AA (the most common pat- varies according to host immunity, the degree of tern), (the complete absence of inflammatory response and the type of hair inva- terminal scalp hair), and (the sion by the . The key feature is patchy total loss of terminal scalp and ) [13]. hair loss with various degrees of Less frequent patterns of AA include the reticu- and scaling and the easy removal of hairs from lated pattern, type, sisaipho type and dif- the affected area [19]. A trichoscopic hallmark of fuse thinning over a part of or the total scalp [16, this disease is the presence of comma hairs. Fun- 17]. Another variant, acute diffuse and total alo- gal potassium hydroxide preparations and cul- pecia, has been described and is characterized by tures and Wood’s lamp are also helpful in estab- rapid progression and extensive involvement lishing the diagnosis of tinea capitis. Oral anti- along with a good prognosis. fungal treatment is required to treat this disease. The typical appearance of AA is of a well-de- The aim of treatment is a clinical and mycologi- marcated localized patch that is asymptomatic, cal cure [20]. round or oval with a smooth surface. There may TTM is classified as an impulse control disor- be single or multiple with no associated der and is a self-inflicted compulsion or habit-tic epidermal changes, such as scaling. In some cases, to pull or pluck at the hair. It is characterized by slightly reddened can be present. AA can af- single or multiple patches with the presence of

Hair Loss in Children 57

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM broken hairs of different lengths. TTM can be ment of this disease and are the first-line choice more difficult to differentiate from AA [21] (see of many dermatologists. Traumatic Alopecia for more information). Topical may be applied pain- TNHL, which is also known as ‘neonatal oc- lessly and have benign side-effect profiles [1]. cipital alopecia’, appears as a bald patch on the Topical potent fluorinated corticosteroids re- occipital region. Initially, it was thought to be sec- main an acceptable form of treatment for chil- ondary to friction due to babies sleeping in the dren with AA but have been reported to have supine position. According to a prospective study, some effects [19, 24, 25]. Children younger than 20% of neonates are born with an observable de- 10 years of age with AA of recent onset tend to ficiency of the occipital scalp hair. This entity can be the most responsive [19]. If hair growth oc- be present at birth and is related to the physiology curs, this treatment should be continued with of hair shaft shedding. TNHL appears in healthy regular monitoring to prevent cutaneous atro- babies from birth until approximately the second phy [26]. Although rare, the development of sys- month of life without accompanying symptoms temic effects must also be monitored in chil- and with spontaneous resolution [2]. It is impor- dren. Relapse after discontinuation of treatment tant to inform parents of the benign course of could happen [27]. Injectable TNHL and its absence of a relationship with the acetonide can be useful in children with patchy sleeping positions of babies. hair loss. Children younger than 10 years of age Congenital triangular alopecia is also known are not usually treated with intralesional corti- as temporal triangular alopecia or Brauer costeroids because of the pain caused by its in- [22, 23]. It is a localized, congenital, nonscarring jection [16]. Older children (>10 years) may be form of alopecia that might be present at birth or prepared to consider this treatment for limited acquired during the first 10 years of life. Typical areas of hair loss if they are able to tolerate the lesions are triangular or lancet shaped, can be injections [19]. unilateral or bilateral, are several centimeters in Systemic corticosteroids have been considered width and are confined to the front temporal re- in the treatment of widespread AA or AA that is gion [23]. Apparently, the lesions are hairless, al- refractory to other local treatments, but careful though very fine vellus hairs may be seen. Trichos- reviews of the protocols (doses, lengths of treat- copy can aid in the differential diagnosis of this ments and side effects) are mandatory. disease by the observation of normal follicular Topical is widely used in children openings containing vellus hairs and the absence and adults for the treatment of AA. Systemic ab- of specific features associated with AA [7]. sorption of the drug is typically minimal with top- There is no consistently reliable treatment for ical therapy. Although side effects are rare, they AA. Its natural history is uncertain, and sponta- have to be taken in consideration, especially in neous remission occurs in some patients. young children. According to Herskovitz et al. Depending on the extent of involvement and [28], a 2-year-old male patient developed general- the patient’s age, watchful waiting is often a sen- ized after 2 months of treatment sible approach. In the majority of children with with 5% minoxidil foam for AA. This report high- patchy AA, hair will regrow entirely within 1 year lights the risk of serious cutaneous or systemic without treatment. The aim of therapy is cosmet- side effects due to the possibility of the systemic ically acceptable hair regrowth. absorption of topical minoxidil. Therapeutic options available for AA in chil- Other treatments for AA include topical im- dren are more limited than for adults. Topical munotherapy (topical agents that induce hair corticosteroids are commonly used for the treat- growth by provoking allergic contact ,

58 Alves · Grimalt

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM such as dinitrochlorobenzene, dibu- Disturbances of Hair Cycle tylester, and diphencyprone), anthralin (induces inflammatory irritant dermatitis), phototherapy, Anagen Hair Loss: and Loose immunosuppressants and immunomodulators Anagen Syndrome [1]. Widespread forms of AA are generally more The undergoes periods of cyclic difficult to cure, and no treatment appears to pre- growth in the following phases: the anagen or vent relapse. growth phase, the catagen phase, the telogen An earlier onset of AA may be indicative of a phase and the shedding exogen phase. At any giv- more widespread disease, although it is impossi- en time, 85–90% of follicles are in the anagen ble to predict its severity. In patients with few phase. During this phase, mitotically active ma- patches of alopecia, prognosis is generally good, trix cells in the hair bulb differentiate and divide, and regrowth might occur within 6–12 months. resulting in hair growth [32]. The duration of the Indicators of a poor prognosis are the presence of anagen growth phase of scalp hair varies from 2 immune disease, , a family history of AA, to 6 years [1]. About 1% of scalp hairs are in the young age at onset, nail involvement, extensive catagen phase, which lasts about 3 weeks. Ap- hair loss and an ophiasis pattern [17]. The course proximately 10% of follicles are in the telogen of AA is unpredictable, and response to treatment resting phase, which lasts about 3 months, after can be changeable. which these hairs are shed. The final step of the hair cycle, exogen, is when the hair is released from the follicle. Anagen loss results from the Androgenetic Alopecia in Children shedding of a large amount of hairs during the anagen phase. The daily loss of telogen hairs is Androgenetic alopecia (AGA) is a nonscarring, considered normal, but if a loss of anagen hair oc- patterned alopecia with a typical pattern of dis- curs, potential causes should be promptly ana- tribution and with genetic involvement [29]. lyzed. The causes that affect children are the same Family history predisposes individuals to the as those affecting adults, and a similar evaluation early development of this disease and rapid pro- should be performed. gression of the alopecia. The onset of AGA is gradual, slowly developing over a period of years. Anagen Effluvium For its development, the presence of In anagen effluvium, hair loss is profound be- is necessary in combination with genetically sus- cause up to 90% of scalp hair is normally in ana- ceptible hair follicles. The onset of AGA is not gen. Hair loss characteristically occurs within 1–2 expected to occur in children without abnormal weeks of the insult. androgen levels [30]. If a healthy prepubertal The most frequent and easily recognizable child presents with AGA, an endocrine evalua- cause of anagen effluvium is radiotherapy and tion is strongly recommended [30]. AGA is not systemic (e.g. doxorubicin, cyclo- uncommon in adolescents and should be consid- phosphamide, vincristine, or bleomycin) [33]. ered as a cause of hair loss. Minoxidil topical so- Other causes that must be considered are expo- lution appears to be effective and well tolerated sure to toxic agents, such as mercury and colchi- in adolescent boys and girls with AGA [31]. Fin- cine, boric acid intoxication (e.g. exposure or in- asteride has not been studied in the treatment of gestion of household pesticides), and ingestion of males younger than 18 years of age; thus, its safe- certain plants (Lecythis ollaria and Leucaena ty and efficacy in adolescents with AGA has not glauca) [34]. Severe protein malnutrition can also been determined. lead to anagen effluvium.

Hair Loss in Children 59

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM Diagnosis is easily made according to the pa- nesses, endocrine disorders, nutritional disorders tient’s clinical history and the presence of dystro- (protein-calorie malnutrition, , or phic anagen hairs. If the insult is removed, nor- starvation), severe emotional stress, immuniza- mal hair regrowth is expected because the hair tion (following bivalent human papillomavirus) cycle was only temporary interrupted. [39] and . Acute oc- curs approximately 3 months after a triggering event. Chronic telogen effluvium is considered In loose anagen syndrome, the anchoring of when shedding continues longer than 6 months. growing anagen hairs to follicles is impaired, with A detailed history should be sought, including a a lack of adhesion of the hair shaft to the hair fol- thorough drug history, and full clinical examina- licle [19]. Because the growing anagen hairs are tion should be performed [40]. A hair pull test is not anchored normally, they can be easily and positive when a high percentage of telogen hairs painlessly plucked from the follicle. is obtained (>20%) [5]. This test should be per- Children between 2 and 7 years of age are the formed at the vertex, parietal and occipital areas most frequently affected with this syndrome. At [33]. birth, the hair is sparse but appears normal, with Telogen effluvium is less frequent in children no fragility or breakage [35]. Around 2–3 years of than in adults, and in children, it is more likely to age, the hair becomes unruly and remains so until be related to sudden disease or trauma. This con- it spontaneously becomes normal at 5–7 years. dition is managed by the treatment of the under- The hair of affected children does not grow lying disorder with appropriate replacement long, and parents commonly state that there is no drugs or medical therapy [19]. The prognosis of need for a haircut. Using light microscopy, hairs telogen effluvium is very good if the precipitating with distorted anagen roots without inner root event is eliminated. sheaths and ruffled cuticles can be observed [35]. Trichogram analysis reveals a preponderance of anagen hairs (>97%) [36]. Traumatic Alopecia During childhood, gentle handling decreases hair shedding. No treatment is necessary because Trichotillomania the hair spontaneously reverts to normal around TTM is characterized by repetitive and self-in- puberty, although some physicians have reported duced compulsive hair pulling [41]. The most fre- the use of a 5% minoxidil solution causing clinical quent site is the scalp, but it can also involve the improvement [37, 38]. eyebrows, and . During in- fancy and early childhood, TTM is more frequent Telogen Effluvium in boys, while during puberty and in adults, there Telogen effluvium is an abnormality of hair cy- is a strong female predominance [42]. cling that can occur at any age. It is a reaction pat- Clinically, patients present with areas of alope- tern in which a percentage of hairs move prema- cia of different shapes, irregular borders and the turely from anagen to telogen, resulting in a dif- presence of hairs of different lengths [21]. This fuse increase in hair shedding. This shedding condition is usually a nonpermanent form of alo- occurs in response to a pathologic or physiologic pecia, but if the same area is persistently plucked, alteration in the health condition. scarring may result with persistent hair loss [1]. The main causes of telogen effluvium are high Typically, there is no scaling on the scalp, al- fever, surgery, drugs (allopurinol, colchicine, be- though some excoriations can be seen. Hair den- ta-blockers, or antihypertensives), systemic ill- sity is normal, and the pull test is negative [5].

60 Alves · Grimalt

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM Trichoscopy reveals the presence of flame hairs Clinical manifestations include hair thinning, (specific for TTM) with the v-sign and tulip hairs, focal decreases in hair density and perifollicular which are highly characteristic features of TTM erythema [45]. Short, broken hairs, [43]. and follicular may be seen [1]. With pro- Three subsets of individuals affected by this longed traction, perifollicular scarring and cica- condition appear to exist as follows: preschool age tricial alopecia develop, which are more evident children, preadolescents to young adults, and along the hairline. During its early stages, alope- adults. In young children, TTM is usually a habit cia is initially reversible with a change in hairstyl- comparable to thumb sucking, and it normally has ing methods. If patients maintain continuous a self-limited and benign course of hair pulling traction, permanent hair loss occurs due to hair with regrowth of the hair if the condition is man- follicle loss. aged conservatively. Several reviews of childhood TTM have related its onset in young children to stressful situations. This type of traumatic alopecia Hair Shaft Disorders is more frequent in preadolescents to young adults with an average age of onset of between 9 and 13 Hair shaft disorders can be inherited or acquired years [21]. These patients tend to have more and sometimes can aid in diagnosing an underly- chronic and relapsing courses of hair pulling. ing disease. A reliable diagnosis of a hair shaft ab- TTM in adults may be secondary to underly- normality can only be made via an evaluation of the ing psychiatric disturbances and has a more pro- wide structural variations found in normal hair. longed course. Hair shaft disorders can be divided in two sub- Management of this disorder is difficult and is categories as follows: (1) hair shaft abnormalities usually based on a person’s age. No specific treat- WITH increased fragility and breakage; and (2) ment has been established that is completely ef- hair shaft abnormalities WITHOUT increased fective. Preadolescents to young adults without fragility. Table 1 has a description of the principal associated psychiatric conditions may benefit hair shaft abnormalities. from nonpharmacological interventions, such as behavior modification programs. If TTM is asso- ciated with psychological/psychiatric disorders, Special Disorders with Hypotrichosis in referral to a psychiatrist for evaluation or treat- Children ment is recommended. Pharmacological inter- ventions include serotonin-specific re-uptake in- There is an extensive list of genetic disorders as- hibitors, tricyclic antidepressants and more re- sociated with hypotrichosis. The detailed analysis cently, N-acetylcysteine, a glutamate modulator of these syndromes falls outside of the ambit of [44]. this chapter.

Traction Alopecia Congenital Atrichia and Hypotrichosis , a pattern of alopecia caused by Atrichia congenita is a rare form of irreversible physical trauma, is induced as a consequence of alopecia with an autosomal recessive mode of in- constant traction while hairstyling (e.g., tight po- heritance that is usually associated with a muta- nytails, or rollers). It is more frequent tion in the human hairless gene located on chro- in black women and children. -textured hair mosome 8 [55, 56]. is particularly easily damaged by such proce- This condition is characterized by follicular dures. agenesis or programmed follicular destruction.

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Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM Table 1. Hair shaft disorders

Disease Genetic/clinical features Diagnosis Associated diseases Management

Hair shaft abnormalities WITH increased fragility and breakage Trichorrhexis Congenital or Acquired (++) Light microscopy: Mental retardation Change in nodosa Acquired TN is frequently due to – nodes along the hair shaft that Argininosuccinic aciduria hairstyling­ [46–48] weathering due to external causes, cause hair to break easily; distal Muenke syndrome procedures­ such as shampooing, styling, UV nodes generally indicate hair Defective teeth and nails Sun hair radiation, wetting and natural weathering; and the absence of Its presence in infants or protection­ friction cuticle cells young children should Hairs appear dry and brittle with a Trichoscopy: ­trigger a search for an tendency to break at different lengths – light magnification: nodular underlying metabolic Three variants of acquired TN: thickenings along hair shafts problem Proximal ATN: seen in patients after that appear lighter in dark hair years of shafts Distal ATN: acquired, cumulative – high magnification: small fibers cuticular damage with an appearance suggestive Circumscribed ATN: scalp, of the ends of two brushes ­ or aligned in opposition Congenital (AD): in the Light microscopy: hairs possess a No specific (beaded hair) hair cortex-specific genes beaded appearance treatment­ [1, 49, 50] KRT86(++), KRT81, KRT83, and DSG4 Trichoscopy: , glycolic Clinically, there is normal hair at – uniform elliptical nodes and acids and birth, but within the first months of intermittent constrictions of ­minoxidil may be life, a characteristic moniliform the hair shaft, causing variation helpful in some aspect of the hair shaft develops. in hair shaft thickness cases Perifollicular erythema and – hairs are bended regularly at There is a wide ­follicular are multiple locations and have a spectrum of common­ features tendency to fracture at severity, and it The hair is short, fragile, brittle and constriction­ sites tends to remain breaks spontaneously or as a result – periodic constriction of the hair constant of friction shaft throughout life In its mildest forms, only the scalp is involved. In extensive cases, there may be , and nail involvement Congenital (AD (++)/AR) or Light microscopy: twists at Björnstad syndromes (pili No treatment [50, 51] ­acquired: isolated or associated ­irregular intervals along the shaft torti associated with May improve in with other conditions Trichoscopy: hairs are twisted and ­sensorineural hearing loss) puberty At birth, the hair is normal. Changes rotated at 180°. In a field of view, Muenke disease gradually appear between the 3rd only part of the hair usually Bazex-Dupré-Christol’s month and 3rd year. More common appears abnormal. Twists are syndrome in girls with blond hair better seen with dry trichoscopy Crandall syndrome Clinically, hairs are fragile, with a using high magnification The presence of pili torti, twisted and spangled appearance requires further evaluation for neurological and ectodermal­ disorders Trichorrhexis Congenital (AD): mutation in the Light microscopy: Marker for Netherton’s No specific invaginata SPINK5 gene ­intussusceptions of the distal syndrome (AR; triad of treatment­ (bamboo Clinically, the hair is short, brittle, shaft into the proximal­ shaft, , atopic diathesis Avoidance of hair) [50–52] sparse and very fragile. May affect with a bamboo appearance and trichorrhexis trauma the scalp, eyebrows and body hair Trichoscopy: golf tee hair (scalp invaginata)­ Defective cornification of the cortex and eyebrow)

62 Alves · Grimalt

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM Table 1. Continued

Disease Genetic/clinical features Diagnosis Associated diseases Management

Trichothio- Congenital (AR), characterized by Light microscopy: irregular, BIDS or Tay’s syndromes No treatment dystrophy sulfur-deficient hair ­undulating contours and (brittle hair, intellectual currently Clinically, only the hair may be ­transverse fractures throughout impairment, decreased available­ affected (fragile) or it may be the hair shaft (‘tiger tail’) fertility, and short stature) Sun protection ­associated with ichthyosis, Trichoscopy: non-specific IBIDS (ichthyosis and BIDS) ­photosensitivity (50%), decreased Diagnosis is performed by PIBDS syndrome growth, and mental handicap sulfur and/or amino acid (photosensitivity­ and analysis of the hair IBIDS) Hair shaft abnormalities WITHOUT increased fragility Rare Light microscopy: hair shafts with No hair or systemic No treatment (ringed hair) Congenital (AD) or sporadic: alternating bright (normal hair) abnormalities­ currently [46, 53] ­abnormalities appear at birth or and dark bands (abnormal hair; available­ during infancy the medulla is expanded by air Aesthetic defect Clinically, the hair looks shiny but cavities within the hair) otherwise normal, and 20–80% of Trichoscopy: hair shafts with the hair is affected regular light bands No fragility, and the hair can grow ­corresponding with dark cavities, long visible by light microscopy Woolly hair Three types of wooly hair: Light microscopy: ovoid cross Hereditary wooly hair: No specific syndrome Hereditary wooly hair (AD): sections, 180-degree longitudinal­ palmoplantar­ keratoderma treatment­ [46, 50] ­ in plakoglobin genes twisting, and cardiac abnormalities Can improve with and desmoplakin genes. Hair color and pili annulati Familial woolly hair: age is variable Trichoscopy: Crawling snake with associated­ with Familial woolly hair (AR): mutations short wave cycles and broken hypotrichosis­ in the LPAR6 and LIPH genes. Hair is hairs Wooly hair nevus: sparse, thin, and short ­associated with linear Wooly hair nevus: localized epidermal or pigmented Clinically, hair is fine and tightly nevi (>50%) curled. Occurs in Caucasians Uncombable Congenital (AD): hair abnormalities Light microscopy (gold standard): Absence of systemic Improves hair (pili appear around the age of 3 years in more than 50% of hairs, there abnormalities­ spontaneously­ trianguli and Clinically, the hair is unruly, dry and is a triangular or reniform shaft with aging canaliculi) impossible to control with a brush with a longitudinal groove or [46, 54] or comb flattening Trichoscopy: triangular or ­reniform hair shaft

TN = Trichorrhexis nodosa; AD = autosomal dominant; AR = autosomal recessive; DSG4 = desmoglein 4; LIPH = lipase H.

Hair at birth can be sparse (or inexistent) and in association with a number of rare syndromes. progress to a total and permanent absence of To be distinguished from AA totalis, a scalp bi- scalp hair over the first 5 years. In another variant opsy may be required. of the disease, neonates are born with lanugo hair, Congenital hypotrichosis is a less severe form which is shed during the first few months of life of atrichia congenita, in which hair is not absent and is never replaced [19, 50]. Congenital atrichia but is diffusely thinned [19]. There is a profound may occur, either as an isolated phenomenon or reduction in the number of hair follicles on the

Hair Loss in Children 63

Ioannides D, Tosti A (eds): Alopecias – Practical Evaluation and Management. Curr Probl Dermatol. Basel, Karger, 2015, vol 47, pp 55–66 (DOI: 10.1159/000369405) Downloaded by: UCONN Storrs - 5/23/2015 4:42:42 PM scalp, which usually but not always is present lashes are also affected. These patients should be from birth. Hypotrichosis may not be noticed examined thoroughly, with particular attention until the age of 2 years because of variation in paid to other ectodermal structures. the quality and quantity of the hair normally present at birth, and it usually occurs as an iso- lated defect. Conclusion

Marie-Unna Hereditary Hypotrichosis Hair problems in children occur relatively fre- Marie-Unna hypotrichosis is an autosomal dom- quently in a wide range of conditions that may be inant condition, which was first described in 1925 congenital or acquired. Not all congenital or he- by Dr. Marie Unna in 27 affected members of a reditary hair disorders are present at birth, and family over seven generations [50]. This rare hy- those that are often go unrecognized. Usually, potrichosis is characterized by isolated progres- these hair disorders are isolated abnormalities, sive alopecia without abnormalities of the nails or and the child is otherwise well. A number of dis- teeth or intellectual or gross motor development. orders occur as part of a multisystem syndrome; Typically, this diffuse hair defect occurs as an iso- thus, recognition of the clinical presentation may lated phenomenon, although some reports have enable the diagnosis of a particular syndrome. If described of its associations with Ehlers-Danlos a disease is hereditary, genetic counseling should syndrome and juvenile macular degeneration. At be offered. A detailed clinical history accompa- birth, hair may be normal or sparse. Around the nied by examination are essential for the accurate third year of life, hair becomes coarse and twisted, diagnosis of the pathology. resembling an ‘ill-fitting ’ [19, 57]. During pu- Hair is very important, both cosmetically and berty, the hair is gradually lost from the crown, functionally. Alterations in the normal appearance ultimately resulting in complete baldness due to of hair can predispose individuals to low self-es- the destruction of the follicles with scarring [19]. teem and a negative body image. Pediatric hair dis- Lashes, eyebrows and body hair are also sparse or orders causes psychological and emotional stresses absent. in both children and parents. It is important that parents are provided a clear understanding of the Ectodermal Dysplasia etiology and natural history of the disease. Ectodermal dysplasia represents a heterogeneous The management of a hair loss disorder should group of inherited disorders affecting more than be adapted according to the age of the patient. An one ectoderm-derived tissue, leading to abnor- early identification of the pathology leads to bet- malities of the hair, nails, , teeth and ec- ter treatment results, including psychological and crine glands. Scalp hair is usually fine and short cosmetic support. Unfortunately, for many hair but silky in texture [19]. Eyebrows and/or eye- disorders, there is no effective, reliable therapy.


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Rubina Alves, Specialist of Dermatology Universitat Internacional de Catalunya Carrer Major 16, 1r ES–08221 Terrassa, Barcelona (Spain) E-Mail [email protected]

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