Primary Generalized and Localized Hypertrichosis in Children

STUDY Primary Generalized and Localized Hypertrichosis in Children

Roopal A. Vashi, MD; Anthony J. Mancini, MD; Amy S. Paller, MD

Objective: To review the causes, presentation, and sets of hypertrichosis cubiti, anterior cervical hypertri- therapy of primary generalized and localized symmetri- chosis, posterior cervical hypertrichosis, and faun tail cal hypertrichosis in children. deformity. All patients with generalized hypertrichosis manifested the condition at birth; the age of onset in chil- Design: Retrospective medical record review. dren with localized symmetrical primary hypertrichosis ranged from birth to 4 years. One girl with generalized Setting: Academic specialty referral clinic for pediatric hypertrichosis had gingival hyperplasia and the girl with dermatological disorders. faun tail deformity had bony diastematomyelia with spina bifida occulta. The medical histories and physical ex- Patients: Case series of 11 prepubertal male and fe- amination findings of all of the children were otherwise male patients who had idiopathic hypertrichosis be- unremarkable. All patients were referred for diagnostic tween July 1, 1990, and November 30, 1999. and therapeutic considerations.

Interventions: None. Conclusions: Primary hypertrichotic conditions, whether localized or generalized, are rare in pediatric patients and Main Outcome Measures: Clinical distribution of in- of unknown origin. Although otherwise benign, these dis- creased hair growth and types of hair removal methods orders may result in cosmetic disfigurement and psy- used. chosocial trauma for patients and families. Patients and their families should be adequately advised of the avail- Results: Seven girls and 4 boys, ranging in age from 4 able treatment methods for both temporary and perma- months to 11 years, were evaluated. Four patients showed nent hair removal. generalized hypertrichosis. The other 7 patients had localized symmetrical hypertrichosis, representing the sub- Arch Dermatol. 2001;137:877-884

YPERTRICHOSIS IS a condi- Primary localized symmetrical areas of hy- tion of excessive hair pertrichosis may occur as hypertrichosis growth that must be dis- cubiti (elbows), anterior cervical hyper- tinguished from hirsut- trichosis, posterior cervical hypertricho- ism, which is character- sis, or faun tail deformity. We evaluated Hized by an androgen-dependent hair the presentation and course of pediatric pattern with excessive body and facial ter- patients with primary symmetrical hyper- minal hair distributed in a male pattern.1 trichosis referred to a specialty dermatol- Hypertrichosis in prepubertal children, un- ogy clinic during a 9-year period and re- like hirsutism, is not associated with an viewed representative examples of these underlying endocrine disorder and is most unusual hair disorders. commonly an adverse effect of drug ad- ministration. Primary hypertrichosis has REPORT OF CASES been classified based on the age of onset (congenital or acquired) and the extent of CASE 1 From the Division of distribution (localized or generalized Dermatology, Children’s 2,3 Memorial Hospital (Dr Paller) forms). A 6-year-old girl was born with long, and the Departments of In its generalized form, hypertricho- coarse, dark brown hair on her forehead, Pediatrics and Dermatology, sis may be an isolated finding, as in hy- with the appearance within months of in- Northwestern University pertrichosis lanuginosa congenita, or as- creased hair on the cheeks, sideburn ar- Medical School, Chicago, Ill. sociated with gingival hypertrophy. eas, chin, chest, abdomen, back, arms, and

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 growth, but was very painful, costly, and only limited areas could be treated at each session. The patient also had SUBJECTS, MATERIALS, AND 8 dental restorations and 2 extractions to preserve her METHODS teeth and dental function. Tonsillectomy and adenoid- ectomy was performed at age 4 years for obstructive sleep apnea. The patient continues to be markedly distressed A retrospective medical record review was per- about her cosmetic problem and refuses to participate in formed of children examined for excessive hair growth activities that expose her, such as swimming. during a 9-year period from July 1, 1990, through November 30, 1999, at the Division of Dermatol- ogy, Children’s Memorial Hospital, a tertiary care cen- CASE 2 ter serving the Chicago metropolitan area of approximately 3 million people. Eleven patients with primary A 7-year-old girl had excessive, fine, long vellus blonde generalized or symmetrical localized hypertrichosis hairs on the back, shoulders, arms, legs, and sacrum. were identified; all had medical records and photo- The increased hair was first noted shortly after birth graphs available for review. Through telephone and and had been progressively increasing in density. The at least 1 clinic-based interview, the following data patient was upset because her classmates teased her and were collected: sex, age, race, medical history, drug called her “werewolf.” There was no reported family history, distribution of increased hair growth, pa- history of excessive hair growth. No associated abnor- tient’s age of onset of excess hair growth, change in pattern of hair growth, family history of increased hair malities were found on physical examination, leading to growth, and treatments attempted. Patients with asym- the diagnosis of hypertrichosis lanuginosa congenita. metrical increased hair growth, with or without an The patient tried shaving and chemical depilatories, but underlying lesion (eg, nevoid hypertrichosis, con- was discouraged about the transient effect. Her parents genital nevi, or smooth muscle hamartoma), and pa- agreed that electrolysis be considered but only when tients in whom hirsutism had been diagnosed based the patient was old enough to tolerate the potential dis- on clinical pattern and endocrine studies were ex- comfort of this procedure. cluded from this review. At follow-up, the patient was 17 years old. The hypertrichosis has persisted and remained the same in density and thickness. The patient was still battling with cosmetic and psychological issues related to the excessive legs. The underlying skin was normal with no associ- hair growth. She had tried electrolysis, which decreased ated pigmentary abnormalities. The patient’s maternal the excessive hair density but was both painful and ex- great-uncle also reportedly had generalized hypertricho- pensive. The patient’s family was unable to continue sis. Although originally seen at Children’s Memorial Hos- therapy after their insurance company denied payment, pital at 10 months of age and diagnosed with general- and they could not afford epilative laser therapy. The pa- ized hypertrichosis, the patient was first noted to have tient uses selective application of chemical depilatory gingival fibromatosis between 12 and 18 months of age, agents that cause skin irritation and must be repeated suggesting the diagnosis of gingival fibromatosis and weekly. hypertrichosis. Examination showed generalized hypertrichosis with RESULTS rough, dark hair, coarse facies with large ears, ante- verted nostrils, micrognathia, hypoplastic midface, gin- Seven girls and 4 boys with hypertrichosis were evalu- gival hypertrophy with dystrophic teeth, pectus excava- ated during the study period (Table 1). Four of the tum, and a protuberant abdomen without organomegaly. children had generalized hypertrichosis; the onset in all The patient had a history of obstructive sleep apnea and of these patients was at birth. Three of the 4 patients had delayed motor skills. Despite the early diagnosis, the no associated medical problems and were diagnosed as patient’s parents sought the opinions of several special- having hypertrichosis lanuginosa congenita (Medelian ists, which led to extensive endocrinologic testing. The Inheritance of Man [MIM] classification: 145700, complete blood cell count, electrolyte levels, urinalysis, 307150). Two of these patients had blonde hair; 1 had thyroid study results, luteinizing hormone level, follicular- brown hair. The hair was soft and silky in all 3 patients. stimulating hormone level, 5-␣ reductase concentra- The fourth patient with generalized hypertrichosis had tion, free testosterone level, total testosterone level, dark brown hair that was coarser than that of the other 3 dihydrotestosterone level, and sex hormone–binding patients with hypertrichosis lanuginosa congenita globulin level were normal. Chromosome analysis showed (Figure 1). In addition, she had associated gingival a 46,XX genotype. hyperplasia (Figure 2), with onset between age 12 and The patient suffered enormous emotional distress 18 months, and developmental delay, suggesting the from the increased hair growth, especially on her face. diagnosis of gingival fibromatosis and hypertrichosis She reported being teased at school and stared at in pub- (MIM: 135400). Of these 4 children, only the girl with lic. Bleaching agents caused minimal improvement in ap- gingival fibromatosis had an affected family member. pearance. A trial of depilatory agents resulted in the re- Her maternal great-uncle reportedly had generalized turn of long, thick, dark brown hairs within 1 week. A hypertrichosis, but the family was unaware of associated trial of epilative intense pulsed light laser therapy on the gingival hyperplasia. The patient’s great-uncle was back at 5 years produced positive results with less hair unavailable for examination.

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Patient No.

Variable 1234567891011

Age at 10 mo 7 y 7 y 10 y 31⁄2 y6y4y9y11y4mo4y presentation Age at onset Birth Birth Birth Birth 2 y 2-3 y Birth 2 y 4 y Birth Birth SexF F M F FFMMF MF Family Hispanic White White White White White White White White White Middle Eastern background (maternal), Swedish- German (paternal) Hair color Brown Brown Blonde Blonde Brown Brown Brown Brown Brown Blonde Black Distribution Generalized Generalized Generalized Generalized Cubiti Cubiti Anterior Anterior Anterior Posterior Sacral cervical cervical cervical cervical Associated Gingival None None None None None None None None None Diastematomyelia abnormalities hyperplasia and spina bifida occulta Affected family Maternal None None None Unknown None None None None None Uknown great-uncle Treatment Chemical Electrolysis Trimming Chemical Trimming Trimming None None Chemical Trimming None depilatory and dipilatory depilatory and intense chemical pulsed light depilatory

Figure 1. Patient 4. Generalized growth of excessive coarse terminal hair as Figure 3. Patient 6. Localized downy growth of increased hair over the shown on the back. dorsal elbows region with hypertrichosis cubiti.

Figure 2. Patient 4. Gingival hyperplasia as a feature of generalized Figure 4. Patient 8. Tuft of luxuriant hair growth on the anterior neck hypertrichosis with gingival hyperplasia. Note the extensive facial hair growth. (anterior cervical hypertrichosis).

The other 7 children had localized hypertrichosis, tail deformity (1 patient) (Figure 5). None of the chil- distributed as follows: elbow and surrounding extensor dren with localized hypertrichosis had an affected fam- arm (2 patients) (Figure 3), anterior cervical (3 pa- ily member, although 2 of the girls were adopted (one tients) (Figure 4), posterior cervical (1 patient), and faun of them having localized hypertrichosis of the arms and

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 must be differentiated from primary causes of generalized hypertrichosis (for differential characteristics, Table 2). Several drugs are well known to cause significant generalized hypertrichosis, particularly oral minoxidil, diazoxide, phenytoin sodium, and cyclospor- ine. Each drug may show a unique distribution of increased hair growth, for example, minoxidil causes hypertrichosis primarily involving the face, shoulders, and extremities.4 In addition, medications such as oral con- traceptives, systemic corticosteroids, and occasionally other drugs such as psoralens and streptomycin sulfate, may increase hair growth, although not to the extent usually seen with minoxidil, diazoxide, phenytoin, and cy- Figure 5. Patient 11. Faun tail deformity on the sacral back area overlying closporine. Discontinuation of drug use leads to even- diastematomyelia and spina bifida occulta. tual resolution of this cosmetic adverse effect. Should drug regimen discontinuation not be an option, common methods of hair removal such as depilatory agents and shav- the other the faun tail deformity). The parents of the ing may be used. 4-year-old girl with the faun tail deformity had never Children with generalized hypertrichosis, known as been counseled about the risk of associated underlying hypertrichosis lanuginosa congenita (MIM: 145700, spinal cord and/or bony abnormality. Magnetic reso- 307150), have a remarkable amount of long vellus hair nance imaging and computed tomographic scans were on the entire body surface, sparing only nonhair- performed and showed diastematomyelia with spina bearing areas, including the mucosae, palms, soles, pre- bifida occulta affecting the lower lumbar spine from L2 puce, and glans penis.3,5 The blonde to black hair may through upper sacrum/S1. Neurosurgical intervention be present at birth or develop during infancy. In some was undertaken. patients, hair will be spontaneously lost during child- The prenatal and perinatal histories of all patients hood; in others, it will remain into adulthood. Associ- were unremarkable; none of the children had been ex- ated abnormalities are rarely described but may include posed to drugs known to cause hypertrichosis. All pa- congenital glaucoma, skeletal abnormalities, and miss- tients were counseled about the available methods of treating teeth.6 Most cases are autosomal dominant,3,7,8 but ing hypertrichosis and their risks. All patients except for there are also reports of X-linked dominant9,10 and au- 2 children with anterior cervical hypertrichosis at- tosomal recessive11 inheritance patterns. tempted treatment. Five of the children chose to use Generalized hypertrichosis has also been associ- chemical depilatories; application in the 3 patients with ated with gingival hyperplasia as a distinct entity. Chil- generalized involvement who used depilatories was lim- dren with gingival fibromatosis with hypertrichosis (MIM: ited to selected areas that caused the greatest cosmetic 135400) generally have excessive body and facial hair in disturbance. One of the children with generalized hy- an identical distribution to hypertrichosis lanuginosa. The pertrichosis used electrolysis selectively to the face and hypertrichosis is often present at birth or develops dur- distal arms. The patient with associated gingival fibro- ing early infancy, but in up to half of the reported cases, matosis tried epilative (EpiLight Hair Removal System; the hypertrichosis begins during puberty.12,13 Some re- EpiLight, Ferndale, Mich) laser therapy but found it to ports have described terminal hair, compared with the be too painful and too costly. The parents considered the lanugo hair of classic congenital hypertrichosis lanugi- suggested treatment to be satisfactory for patients with nosa, in this group of patients.14 Inheritance is autoso- localized involvement but unsatisfactory in all children mal dominant and the pathogenesis is unknown. Some with generalized hypertrichosis. cases have been associated with mental retardation15 and/or seizures.16-19 Gingival hyperplasia is usually noted COMMENTS when teeth fail to appear on schedule and, thus, usually following the observation of hypertrichosis. The gingi- Primary and secondary hypertrichosis in children, par- vae are described as pink, firm, pebbly or nodular in ap- ticularly if extensive, may cause severe cosmetic alter- pearance, and sometimes with “orange peel” stippling. ation, resulting in a tremendous emotional burden. Treat- There are no other associated dermatological features. ment options are limited and the results of therapy Complications in these patients include interference with disappointing. Although primary symmetric hypertri- chewing, respiration, and speech. Failure of the teeth to chosis is rare, it is often confused with hirsutism, lead- erupt may be associated with periodontal abscesses. Pa- ing to evaluation for adrenal gland disorders. We have tients may require surgical debulking of the gingivae to examined the features and courses of children with ex- help preserve teeth and function, but recurrence is in- cessive hair growth unrelated to systemic disorders or the evitable, requiring repeated procedures. use of medications. Four patients had primary general- The 7 other patients described in this article en- ized hypertrichosis and 7 had localized symmetric hy- compass the spectrum of localized symmetrical hyper- pertrichosis (Table 1). trichosis (MIM: 139600, 239840), including hypertri- Acquired generalized hypertrichosis, especially that chosis cubiti, anterior cervical hypertrichosis, posterior related to medication usage, is much more common and cervical hypertrichosis, and lumbosacral hypertricho-

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Primary Generalized Gingival Fibromatosis Primary Primary Cervical Primary Characteristic Hypertrichosis and Hypertrichosis Hypertrichosis Cubiti Hypertrichosis Faun Tail Deformity Synonyms Congenital hypertrichosis Hypertrichosis terminalis, Hypertrichosis cubiti Anterior cervical Lumbosacral lanuginosa, congenital generalized, with gingival hypertrichosis and hypertrichosis generalized hypertrichosis, hyperplasia posterior cervical hypertrichosis lanuginosa hypertrichosis congenita, and hypertrichosis universalis Hair distribution Entire body surface except Entire body surface except Bilateral elbows Anterior or posterior aspect Lumbosacral the mucosae, palms, mucosae, palms, soles, of the neck soles, prepuce, glans prepuce, glans penis, and penis, and labia minora labia minora Prenatal diagnosis None None None None None Age detectable Birth or early infancy Birth, early infancy, and Birth or early infancy Birth Birth puberty Male-female ratio 1:1 Unknown Unknown Unknown 1:4 Risk of occurrence Rare Rare Rare Rare Rare Treatment Chemical depilatories, Chemical depilatories, Chemical depilatories, Chemical depilatories, Imaging studies electrolysis, laser, electrolysis, laser, bleaching, plucking, waxing, plucking, waxing, shaving, or surgical bleaching, and trimming and trimming; surgical shaving, trimming, trimming, and bleaching intervention; debulking of gingivae and bleaching trimming Type of Primary generalized Gingival fibromatosis and Primary hypertrichosis Primary cervical Primary faun tail hypertrichosis hypertrichosis cubiti deformity Complications Psychosocial difficulties Interference with chewing, Psychosocial Psychosocial difficulties Psychosocial respiration; and speech; difficulties difficulties failure of eruption of teeth; and psychosocial difficulties Inheritance Autosomal dominant Autosomal dominant Unknown Posterior: X-linked recessive Unknown or autosomal dominant; anterior: probably autosomal recessive Possible None Epilepsy and mental Short stature Kyphoscoliosis and Spina bifida occulta, associations retardation peripheral neuropathy diastematomyelia, and tethered cord

sis. In patients with hypertrichosis cubiti or “hairy el- described in association. This developmental defect bows syndrome,” lanugo hairs are present symmetri- often overlies bone and spinal cord defects, most com- cally at birth or develop during infancy on the extensor monly spina bifida occulta and diastematomyelia.28,29 If surfaces of the elbows, extending from midhumerus to undetected and uncorrected surgically, complications midforearm. This uncommon form is usually not asso- may ensue with advancing age. As the vertebral column ciated with other anomalies and most frequently only grows disproportionately with respect to the spinal col- represents a cosmetic problem.20,21 There have been iso- umn, fibrous bands that connect the skin to the spinal lated case reports of hypertrichosis cubiti associated theca pull on the spinal theca and may result in neuro- with short stature.22,23 In patients with anterior cervical logic abnormalities. The most common defects are foot hypertrichosis, the patch of hair is at the sternal notch drop, nocturnal enuresis, and backache, sometimes and lower anterior aspect of the neck. The mode of developing as late as adolescence or adulthood. Com- inheritance is most likely autosomal recessive. puted tomographic and/or magnetic resonance imaging Although usually an isolated finding, anterior cervical myelography are diagnostic. Early surgery is particu- hypertrichosis may be associated with peripheral sen- larly critical with diastematomyelia associated with sory and motor neuropathy (MIM: 239840) as an auto- spina bifida.30 somal recessive trait.24,25 One case report describes a boy with anterior cervical hypertrichosis from early MANAGEMENT OF HYPERTRICHOSIS infancy, associated with developmental delay and peripheral neuropathy.26 In posterior cervical hypertri- Long-term removal of unwanted hair is a challenge. The chosis, the excessive hair overlies the cervical vertebrae. need for treatment depends on the degree of hypertri- It has been described as an X-linked or autosomal chosis and the psychosocial needs of the child. Even in dominant trait. Posterior cervical hypertrichosis has exceedingly hairy newborns and young children, some been associated with underlying kyphoscoliosis (MIM: hair removal may be necessary to alleviate problems the 117850) as an autosomal dominant disorder.27 Lumbo- family and society may have in accepting the child.31-33 sacral hypertrichosis or faun tail deformity is localized The current available treatment methods for removal of to the sacral midline. A variety of concurrent cutaneous excessive hair include bleaching, trimming, waxing, physi- malformations such as sacral dimple, lipoma, hyperpig- cal and chemical depilatories, electrolysis, intense pulsed mentation, port-wine stain, or dermoid cyst have been light therapy, and laser hair removal.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Bleaching is a quick, easy, and painless process that easy, and effective, but the generated hydrogen sulfide removes natural hair pigment partially or totally, light- releases an unpleasant odor and can often irritate the skin. ening the hair to a yellowish hue.34 The active ingredi- Most commercially available chemical depilatories, as a ent is hydrogen peroxide, which softens and oxidizes the result, consist of thioglycolates. Thioglycolates are less hair. Bleaching can last up to 4 weeks. This method is odiferous and less irritating, but take a longer time to act best for use on light-skinned children because yellow than the sulfides. They can be used in more sensitive ar- bleached hair may emphasize the increased hair when eas, such as the face, and work best on fine vellus hair. viewed against the skin of more darkly pigmented chil- Enzymatic depilatory agents do not have the offensive dren. Occasionally, bleaching results in skin irritation. odor and are nonirritating, but they are not effective. Trimming of the hair is a recommended option for The use of chemical depilatories is limited because young children with either localized or diffuse hypertri- of several disadvantages: (1) they are only suitable for chosis. Trimming involved areas will make the hair less small areas, (2) they are associated with a risk of allergic noticeable and will not result in acceleration of hair re- contact dermatitis, (3) their application is messy, (4) they growth. have an unpleasant odor, and (5) they are relatively ex- Physical depilation includes tweezing or plucking, pensive, especially if treating larger areas. The localized shaving, and waxing.35 Tweezing is an effective tempo- irriation is a well-known potential adverse reaction to rary hair removal method, but it is a slow, tedious, and chemical depilatories. Additional adverse effects from painful process. This temporary method only tears off ana- use of thioglycolates, as in permanent wave products gen hair bulbs in varying break patterns allowing for re- and straighteners, include allergic contact dermatitis and, growth of the hair follicles. Complete removal of the fol- with inadvertant eye contact, corneal alkali burns.41-44 licular bulb including both the matrix epithelium and To minimize the risk of local irritation, it is prudent to papilla rarely occurs.36 For childhood hypertrichosis, apply the chemical depilatory to a test site first, adhere plucking may be a feasible option for patients with very to the recommended time limitations, wash the treated localized areas of increased hair growth but too demand- area thoroughly, and follow application with use of a ing for diffuse hair growth. Adverse reactions include hy- moisturizer.45,46 perpigmentation, folliculitis, scarring, ingrown hairs, and Children with more extensive hypertrichosis must distorted follicles.37 The results of depilation can last up limit treatment with chemical depilatories to localized to 4 weeks, but in our experience have been particularly sites because of the additional (theoretical) risk of sys- transient in these patients with hypertrichosis, leading temic thioglycolate absorption and toxic reactions. Data to rapid hair regrowth within a week or two. Shaving does are lacking on the potential adverse reactions from ac- not affect the width or rate of regrowth of individual cidental ingestion of thioglycolates or on systemic toxic hairs,38 but the hairs as they grow out are thicker and more reactions from local application; however, parenteral ad- coarse, without the finer tapered end of unshaven hair. ministration of large amounts of thioglycolates in rats leads Shaving is not recommended because the diameter of the to increased lacrimation, increased intestinal peristal- cut hair at the surface is greater than that of the uncut sis, convulsions, and respiratory failure with death.47-49 tapered hair. As a result, daily shaving must be under- Permanent hair removal methods, such as electroly- taken or the cosmetic result is worsened. Certain areas sis and laser therapy, have not been studied in prepu- of excessive hair, such as the legs, may be amenable to bertal children, and their use has been limited in older daily shaving. Males also have the option of shaving ex- children and adolescents. Both are costly alternatives. Elec- cess hair on the beard and mustache regions, but this may trolysis, a permanent hair removal technique, can be ac- be unacceptable psychologically for women. Although complished by 1 of the 3 following methods: galvanic, dry or electric shaves are not as close as wet shaves, a thermolysis, and blend.37 In galvanic electrolysis, a dry electric razor has been effectively used to treat gen- direct electric current is delivered to the hair follicle eralized hypertrichosis during the neonatal period.33 through an inserted needle. The current produces so- Wax epilation is a painful process for the removal dium hydroxide, which acts as a caustic agent to de- of fine vellus hair. Waxing methods are used with cold, stroy the hairbulb and dermal papilla. This modality is warm, or hot wax. A soft regrowth occurs within 4 to 6 the most effective, but the slowest. In thermolysis, a high- weeks. All areas of the body, excluding genital regions, frequency alternating current produces heat in the fol- can be treated with waxing methods. Waxing causes licular tissue causing destruction of the hair bulb. This greater discomfort and is more expensive than shaving. method is quick, requiring only a few seconds per hair, Other possible adverse effects include skin irritation and but not as effective in thick hairs or in highly curved hair folliculitis.39 Another drawback of this epilating tech- follicles. The blend method combines galvanic electroly- nique includes the requirement for the hair to be a mini- sis and thermolysis; it is the most efficient electrolysis mum length of 2 to 3 mm to be grasped by the wax. There- technique. The best result occurs if the area is shaved sev- fore, no other hair removal methods may be used for eral days before epilation so that only anagen hairs are several days prior to waxing.40 This physical depilatory epilated. Permanent hair removal can occur only if the method is too painful and traumatic for use on children needle is inserted deep into the follicle, enabling the cur- but may be an option for older adolescents. rent to travel to the germinative bulb. Hair is not an elec- Chemical depilatories contain sulfides, thioglyco- trical conductor and cannot transmit an electrical cur- lates, and enzymatic depilatory agents. They break down rent to the hair bulb. Therefore, the commercially hair by cleaving its cysteine linkages35 and cause mini- promoted electric tweezer method does not produce per- mal damage to the underlying skin. Sulfides are quick, manent hair removal.50

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 The main disadvantage of electrolysis is the associ- hair. In these mutant homozygotes, anagen VI, the last ated pain. Although preapplication of ice packs or topi- stage of anagen in which the hair elongates and pro- cal anesthetics, such as a eutectic mixture of 2.5% lido- trudes through the skin, is approximately 50% longer caine and 2.5% prilocaine hydrochloride (Emla Cream; than in heterozygotes or wild-type mice. This mutation Astra USA Inc, South San Francisco, Calif )51 or 4% li- does not affect other stages of the hair cycle or produce docaine (ELA-Max; Ferndale Laboratories Inc, Fern- any structural abnormalities. The angora gene has been dale, Mich) may decrease discomfort, electrolysis is poorly shown to be a mutant allele of fibroblast growth factor tolerated in children. Other adverse effects include tran- 5, a signaling molecule that is localized to the outer root sient postinflammatory erythema and whealing, bruis- sheath of the lower one third of the hair follicle,63 only ing, swelling,52 and, in darker-skinned patients, postin- during mid to late anagen VI. No human homologue for flammatory hyperpigmentation. the angora mouse has yet been found. Keratinocyte Laser therapy and intense pulsed light therapy are growth factor, also known as FGF7, has also been the newest available permanent hair removal sys- shown to stimulate proliferation and differentiation of tems.53-58 These techniques remove unwanted hair through early progenitor cells within hair follicles and sebaceous the selective photothermolysis of melanin-rich struc- glands.64 The absence of FGF7 in a knockout mouse tures, thus, entailing light energy absorption in hair fol- model led to a greasy or matted rough hair coat.65 The licles and minimal absorption by surrounding tissues. This phenotypic result of excessive expression of or muta- method achieves photothermal destruction of hair fol- tions in keratinocyte growth factor in humans is licles while maintaining a low temperature of the sur- unknown. Understanding the molecular nature of the rounding tissue to avoid skin damage. This hair re- signals of the hair growth cycle will be critical to under- moval system seems to be more effective for darker hair. standing the mechanisms for increased hair growth in Treatments result in significant clearance of excess hair, generalized or localized hypertrichosis and may lead to with the neck, chin, and lip as the most common areas novel therapies. of treatment. Occasionally, posttreatment erythema, edema, blisters, and hyperpigmentation occur. Lasers ca- Accepted for publication December 26, 2000. pable of hair removal include ruby, diode, Nd:YAG, and Corresponding author: Amy S. Paller, MD, Division Alexandrite.57,58 As with traditional electrolysis, laser elec- of Dermatology, Children’s Memorial Hospital, 2300 trolysis is uncomfortable for children, which limits its Children’s Plaza—107, Chicago, IL 60614 (e-mail: usefulness. [email protected]). A promising novel treatment for retarding excessive hair growth (not removing hair) is eflornithine cream, REFERENCES which irreversibly inhibits ornithine decarboxylase, an enzyme present in hair follicles that is important in hair 1. Barth JH, Wilkinson JD, Dawber RP. Prepubertal hypertrichosis: normal or ab- 59,60 growth. Twice daily application of 15% eflornithine normal? Arch Dis Child. 1988;63:666-668. hydrochloride cream reduced excessive, unwanted fa- 2. Bertolino AP, Freedberg IM. Hair. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freed- cial hair compared with vehicle treatment. Local irrita- berg IM, Austen KF, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill Co; 1993:689-690. tion, characterized by burning, stinging, and/or tin- 3. Sybert VP. Hypertrichosis lanuginosa congenita. In: Sybert VP, ed. Genetic Skin gling, occurred more frequently in eflornithine-treated Disorders. New York, NY: Oxford University Press Inc; 1997:172-175. patients.61 Studies to date have involved limited appli- 4. Earhart RN, Ball J, Nuss DD, Aeling JL. Minoxidil-induced hypertrichosis: treat- cation in adult women; the safety and effectiveness in chil- ment with calcium thioglycolate depilatory. South Med J. 1977;70:442-443. dren have not been established. 5. Cockayne EA. Ectodermal dystrophies. In: Cockayne EA, ed. Inherited Abnor- malities of the Skin and Its Appendages. London, England: Oxford University Press; 1933:245-249. PATHOGENESIS OF HYPERTRICHOSIS: 6. Judge MR, Khaw PT, Rice NS, Christopher A, Holmstrom G, Harper JI. Congen- POSSIBLE CANDIDATE GENES ital hypertrichosis lanuginose and congenital glaucoma. Br J Dermatol. 1991; 124:495-497. 7. Beighton P. Congenital hypertrichosis lanuginosa. Arch Dermatol. 1970;101: The regulation of hair cycling is not completely under- 669-672. stood. The hair growth cycle is defined as having 3 phases: 8. Suskind R, Esterly NB. Congenital hypertrichosis universalis. Birth Defects Orig anagen, the stage of follicular regeneration and growth; Artic Ser. 1971;7:103-106. catagen, the stage in which cell proliferation ceases and 9. Macias-Flores MA, Garcia-Cruz D, Rivera H, et al. A new form of hypertrichosis the hair follicle shortens; and telogen, the stage during inherited as an X-linked dominant trait. Hum Genet. 1984;66:66-70. 10. Figuera L, Pandolfo M, Dunne PW, Cantu JM, Patel PI. Mapping of the congen- which the hair is shed. The abnormal hair growth pat- ital generalized hypertrichosis locus to chromosome Xq24-q27.1. 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