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Editors: Freedberg, Irwin M.; Eisen, Arthur Z.; Wolff, Klaus; Austen, K. Frank; Goldsmith, Lowell A.; Katz, Stephen I. Title: Fitzpatrick's Dermatology in General Medicine, 6th Edition Copyright 2003 McGraw-Hill > Table of Contents > Part Three - Disorders Presenting in the Skin and Mucous Membranes > Section 10 - Disorders of Epidermal Appendages and Related Disorders > Chapter 71 - Hair Chapter 71 Hair Elise A. Olsen As with abnormalities of other organ systems, hair disorders can represent either a primary or secondary dysfunction and can be related to exogenous or endogenous causes. Hair is unique when compared to other organ systems, however, in that it undergoes repetitive planned obsolescence and rebirth, laying open the possibility of clinical disorders based on cycling abnormalities. Further difficulties arise when the type and/or amount of hair in a given body area deviates from the expected norm. The end result of any of these abnormalities, either hair loss or overgrowth, often leads to major psychological problems for patients. This section on hair abnormalities reviews the primary causes of hair loss and hair overgrowth and defines the significant pathophysiologic and clinical features and therapeutic options of each. HAIR CYCLE (See also Chap. 12) Knowledge of the hair cycle is vital to understanding hair problems. The duration and rate of growth of the anagen (growth) phase normally vary at different body sites, in different individuals, and at various ages, and

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determine the ultimate length of hair in that area.1 Catagen is the transitional portion of the cycle between anagen and telogen and is short lived (2 to 4 weeks) in duration. Telogen duration also varies greatly in different body sites, but interindividual variability appears more limited. In a normal scalp, telogen is assumed to last 3 to 4 months and anagen to last 3-plus years. With age, there is both a diminution in anagen duration and an increase in the time interval between two anagen cyc les.2 At any given time, ~90 percent of the scalp hair is in anagen and 10 percent in telogen; this is subject to some seasonal variability.3 At the end of anagen, each hair bulb moves from its location in the subcutaneous tissue or dermis (depth of location is determined by whether the follicle is terminal or vellus) to a more superficial location by means of shrinkage and remolding of that portion of the follicle below the bulge where the arrector pili muscle inserts. The concentric layers

of the inner root sheath, which anchor the hair shaft in the follicle, are only present to the bottom of the isthmus of the follicle, the region to which the hair bulb ascends in telogen (Fig. 71-1). Consequently, the hair shaft in telogen is no longer anchored securely in the tissue, as it was in anagen, and it may be dislodged with the gentle traction of shampooing, combing, brushing, etc. The recapitulation of the anagen follicle and initiation of growth of a new anagen hair leads to the shedding of any remaining telogen hair in the follicular canal (see Fig. 12-5).

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FIGURE 71-1 Diagram of a normal anagen hair follicle. DIAGNOSTIC TECHNIQUES Techniques to Assess Cycling Abnormalities There are several tools for determining aberrations of scalp hair cycling. The first is the hair pull, which should be done on every patient with a complaint of hair loss. This simple technique involves manually grasping a group of 50 to 100 scalp hairs and applying gentle P.634

traction fro m the base to the terminal ends and repeating this in various areas of the scalp.4 Normally, in the author's experience, only three to five hairs total are dislodged on six to eight such hair pulls if the hair has been

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shampooed regularly: shedding of more than three to five hairs per hair pull is pathologic. This is a subjective test and results normally will vary slightly in any given patient depending upon the physician performing the test, each of whom may exert different degrees of traction on different sizes of hair clumps. In the case of increased shedding, the proximal ends of t he hairs so obtained should be evaluated microscopically to determine whether there is an intact hair shaft and bulb, indicating either an effluvium (Latin, a flowing out ) or hair breakage. The hair bulbs should be further evaluated to determine whether they are normal telogen (indicating a physiologic aberration) versus dystrophic telogen or anagen hairs

(indicating a pathologic process). There is little reason to do a hair pluck today. This momentarily painful technique involves applying a cla mp or needle holder to the base of 50 to 100 hairs and quickly pulling the hairs out en masse. Although this technique, which is used in a trichogram, allows one to determine an anagen/telogen ratio by inspection of the proximal hair bulbs, it fails to give critical information about the type of hair being shed. A third means of determining specifics regarding the hair cycle is the phototrichogram.5 In this technique, hairs are clipped very short or shaved in a given target area and comparative photographs are taken of the target area at baseline and again 2 to 3 days later. As only the anagen hairs will have increased their length on subsequent follow -up (normal hair growth is ~ 1 cm/month), this technique can be used to determine (1) the percentage of hairs in anagen based on the number of longer hairs compared to total hairs and (2) with sophisticated cameras and co mputer software, the hair growth rate. To be reliable, a phototrichogram requires locating the same

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precise target site at each time point, standardized photography, and standardized hair counting by image analysis. A related procedure, called a hair window, involves clipping or shaving the hairs in a given area and having the patient return anywhere from 3 to 30 days later to evaluate regrowth. Without photography, this is a gross technique for assessing the potential for regrowth in a given area, and does not yield reliable information regarding the specifics of cycling in the target area. Biopsy A biopsy of the scalp may or may not help with the diagnosis of a particular hair disorder. The information it delivers about cycling

aberrations is merely confirmatory to that obtained by other simpler, less expensive means, and the diagnosis of hair shaft abnormalities cannot be made by a scalp biopsy. The real value of a scalp biopsy is the insight it can offer into mechanisms of alopecia. A 4 -mm punch biopsy is preferred to a 3-mm one because of the ease of laboratory preparation and accumulated quantifying data on normal scalp in the larger specimen. The biopsy should be taken in the direction of the follicle growth and to a sufficient depth to contain the follicular bulbs in anagen (generally into the subcutaneous tissue). Vertical sectioning of the biopsy specimen gives a n immediate overview of the anatomy of the tissue fro m the epidermis down to the fat, but unless multiple step sections are taken, the view is limited to a snapshot of a few follicles. Horizontal (or transverse) sectioning of the specimen gives a simultaneous overview of many follicles.6 This latter technique requires sectioning at several different levels of the skin because the terminal portion of the hair follicles will be at different depths depending on the type (terminal versus vellus) of hair and part of the cycle

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(anagen versus telogen) they are in, and because the pathology may lie anywhere along the length of the follicle. Once appropriate sectioning is done, however, all the follicles in a given horizontally sectioned biopsy ca n be viewed simultaneously, giving a much greater amount of information than is available from a similar number of vertical sections. Hair Shaft Evaluation The preferred hairs to examine in patients with a complaint of shedding are those gently pulled from the scalp. This avoids the two major problems of a hair pluck: physical distortion of the hair shaft by a clamp and the distortion of all anagen hair bulbs so obtained. The proximal portions of the collected hairs should be lined up on a slide in a drop of cyanoacrylic glue, a coverslip applied, and the hairs viewed under light microscopy. Telogen hairs have a cornified rounded-up bulb without an attached root sheath (Fig. 71-2A). Anagen hairs are recognized clinically by their pigmented, somewhat distorted, malleable bulb. P.635

Anagen hairs should not normally be found in a hair pull except in very young childrenin this case, anagen hairs with an attached ruffled cuticle, so-called loose anagen hairs (Fig. 71-2B), may be found in small numbers, probably secondary to poor hair shaft anchoring to the root sheath.7 Comparison with a normal anagen hair obtained by hair pluck is shown in Fig. 71-2C.

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FIGURE 71-2 A. Normal telogen hair obtained by hair pull. B. Loose anagen hair obtained by hair pull; note the ruffled cuticle. C. Proximal end of normal anagen hair obtained by hair pluck. D. Distal ends of newly regrowing anagen hairs; note the tapered tips and comparative dia meter with established anagen hairs. In those in whom the hair appears to be breaking off, not growing, or unruly, the distal portion of the hair shaft should be evaluated. If a fungal infection is suspected, potassium hydroxide (KOH) instead of glue should be placed on the slide and the proximal hair examined for spores and hyphae. The proximal hairs may need to be plucked from the scalp in this case. In other situations, where a hair shaft disorder is suspected, a gentle hair pull should be used to obtain hairs, but if no hairs are forthcoming, then hairs should be cut, not plucked, for evaluation. A newly growing anagen hair will have a tapered distal tip ( Fig. 71-2D) rather than the blunt distal end of hairs that have been cut or trimmed or in which the ends are intrinsically broken. Most hair shaft abnormalities can be diagnosed by light microscopic examination, although some types will require further examination by scanning electron microscopy to confirm findings only suggested by light microscopy (e.g., longitudinal grooving). Polariscopic exa mination should be performed if trichoschisis (a particular pattern of hair breakage) is seen under light microscopy in order to evaluate potential trichothiodystrophy (sulfur-deficient hair). The etiology of brittle hair can be further pursued by direct amino acid analysis of whole-hair hydrolysates and electrophoretic characterization of the main classes of proteins in hair (generally defined by their cysteine

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content and designated ultrahigh-, high-, and low-cysteine or ultrahigh, high-, and low-sulfur proteins). ALOPECIA To begin a discussion of hair loss or aberrant hair growth, it is useful to have a means of organizing, rather than merely cataloguing the myriad causes of alopecia. Deciding whether the hair loss is diffuse (global) or focal (patchy or localized) can facilitate differentiation of the types of alopecia. The following pathophysiologic categories can aid in further defining the diffuse hair loss: (1) failure to produce or continue to produce a normal hair follicle; (2) aberrations in the production of a normal hair shaft; (3) aberration of the normal hair cycle; and (4) destruction of the hair follicle. Determining whether the hair loss is a destructive or a nondestructive process (clinically suggested by observing whether

follicular openings are preserved in areas of hair loss) can further narrow the differential diagnosis. An approach that uses the afore mentioned diagnostic tools to aid in assignment of the hair loss process to one of these categories is given in Table 71-1.

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TABLE 71-1 Differential Diagnosis of Hair Loss

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Diffuse (Global) Hair Loss FAILURE OF FOLLICLE PRODUCTION There has been a recent explosion of knowledge, largely fueled by null mutant rodent models, regarding the genetic causes of diffuse alopecia presenting in infancy and childhood. The most notable finding is the abnormality of the human homologue of the mouse hairless (hr) gene, which causes congenital universal atrichia and atrichia with papular lesions.8,9 and 10 The atrichia may be present from birth or develop over the first year of life: this is concordant with an abnormality in

recapitulation of the anagen follicle after the first pelage as seen in hairless knockout mice. The patients with atrichia with papular lesions develop follicular cysts, generally 3 to 18 years after the alopecia ( Fig. 71-3). The hairless gene product is a putative multifunctional transcription factor and the gene locus is on chromoso me 8p12. A similar clinical phenotype wit h universal hair loss during the first year of life and cutaneous cysts years later but P.636

accompanied by rachitic bones is seen in hereditary vitamin D-resistant rickets.11 This condition is secondary to mutations in the vitamin D receptor, probably in the zinc finger domain, which, like the hr gene, acts as a transcription factor. Patients previously diagnosed with alopecia universalis (alopecia areata) at birth or few months of age may, in fact, have one of the conditions noted above, particularly if the universal hair loss is persistent and/or familial.

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FIGURE 71-3 Atrichia with papular lesions. (Photograph courtesy of Abraham Zlotogorski, MD.) HAIR SHAFT ABNORMALITIES Diffuse hair loss in infancy is more commonly one of hypotrichosis tha n total atrichia and may be secondary to abnormal production of a subset of hair follicles and/or hair shafts. Most of the hereditary alopecias do not occur alone but in the company of a constellation of other anomalies, most frequently bone, central nervous system, or eye. Freire -Maia has suggested a classification system for those hereditary disorders of ectodermally

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derived tissue that demonstrate a primary abnormality of two of the following: hair, teeth, nails, or eccrine glands: these are termed type A ectodermal dysplasias.12 An abnormality of one of these four major anomalies plus one other ectodermal sign is termed type B

ectodermal dysplasia. Those ectodermal dysplasias that involve hair have been further catalogued in the available detail by Olsen using the classification system of type A ectodermal dysplasias.13 Most reported syndromes that involve hair are also discussed and regularly updated, including genetic information, in McKusick's Mendelian Inheritance in Man.14 These three sources facilitate the diagnosis of hereditary childhood alopecias. Hair shaft abnormalities can be primary and hereditary or secondary to external factors. Some hair shaft abnormalities represent common endpoints to various forms of trauma or inherent shaft weaknesses, and some are specific to a particular constellation of findings or inherent make-up of the hair shaft. Hair shaft abnormalities can be divided into those associated with hair breakage and those associated with unruly hair. Hair shaft abnormalities associated with hair breakage TRICHORRHEXIS NODOSA The most common defect of the hair shaft leading to hair breakage is trichorrhexis nodosa.15 Mechanical or che mical damage triggers this response, which can occur in normal hair, but occurs much more read ily in inherently weak hair. Microscopically, the affected hair develops a breach in the cuticle, with eventual separation and fraying of the exposed cortical fibers leading to a nodal swelling. 16 The fibers then fracture and the shaft

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breaks with the resultant appearance of a splayed paintbrush or fanlike array (Fig. 71-4). Trichorrhexis nodosa may be congenital or acquired.

FIGURE 71-4 Trichorrhexis nodosa. Congenital trichorrhexis nodosa may be present at birth or may appear within the first few months of life. Although it might occur as an isolated defect or, rarely, with follicular hyperkeratosis or teeth/nail defects, 15 the occurrence of congenital trichorrhexis nodosa should lead to a search for an underlying metabolic disorder. Patients with argininosuccinic aciduria, primarily those with the late-onset form (occurring at >2 years of age), 15 have associated hair defects. In this condition, in which absence of the enzyme argininosuccinase leads to an accumulation of the nitrogenous waste precursor argininosuccinic acid, brittle lusterless hair develops along with psychomotor retardation and ataxia. The diagnosis is established by

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finding acidosis, hyperammonemia, and low serum arginine.17 Citrulline (the normal precursor of argininosuccinic acid in the urea cycle)

accumulates in the condition citrullinemia, which is caused by a defect in the enzyme argininosuccinic acid synthetase. In this condition, the hair is brittle and both trichorrhexis nodosa and pili torti (another common hair shaft defect) may be present.15,18 Affected infants have an associated dermatitis that may be widespread but more pronounced in the perioral and diaper area. Patients with Menkes syndrome, caused by a defect in copper efflux,19 and trichothiodystrophy, caused by a defect in the synthesis of the ultrahighand high-sulfur proteins integral to hair,20 both have

trichorrhexis nodosa apparent on microscopic examination of the associated brittle hair. Acquired trichorrhexis nodosa may be either distal or proximal. Proximal breakage appears most commonly in African-American women, usually after repetitive che mical or hot-comb straightening.15 Distal trichorrhexis nodosa is more commonly secondary to excessive brushing, back-combing, or sporadic use of permanent waves. Treatment of trichorrhexis nodosa, congenital or acquired, is by avoidance of chemical or physical trauma to the hair. TRICHOSCHISIS The break in trichoschisis is a clean transverse fracture through the entire hair shaft at a location where there is a focal absence of cuticle ( Fig. 715A). Trichoschisis is usually, but not specifically, a marker for the sulfurdeficient hair of trichothiodystrophy, in which the hairs of the scalp, eyelashes, and brows are short and brittle.15 The hair abnormality of trichothiodystrophy identifies a group of autosomal recessive disorders in

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which

acronyms

or

eponyms

identify

particular

constellations

of

extratrichologic neuroectodermal abnormalities (Table 71-2). In affected individuals, the hair cystine P.637

P.638

content is less than half normal, primarily fro m a major reduction and altered co mposition of the ultrahigh-sulfur matrix proteins.20 Polariscopic examination of affected hairs characteristically shows alternating light and dark bands, presumably secondary to variations in sulfur content 15 (Fig. 71-5B). Sulfur and/or amino acid analysis of the hair is diagnostic.

TABLE 71-2 Trichothiodystrophy

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FIGURE 71-5 Trichothiodystrophy. A. Hair shaft with trichoschisis without polarization (light micrograph 100). B. Hair shaft with polarization (light micrograph 100). (From Whiting,15 with permission.) Patients with trichothiodystrophy, particularly the 50 percent with

associated photosensitivity, may have a defect in excision repair of ultraviolet damage but without an increased risk of skin cancer. 21 Recent data support correlation of mutations in the DNA repair and transcription gene ERCC2 locus with the nucleotide excision repair characteristics of both trichothiodystrophy and xeroderma pigmentosa.22 No treat ment is currently available, but photosensitive patients should be tested for their cellular response to ultraviolet radiation and encouraged to practice sun protection.

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PILI TORTI The short and brittle hairs in patients with pili torti, when viewed through a microscope, appear flattened and twisted through 90 to 360.15 The twisting must be differentiated from the normal twisting seen in Negroid hair and in the pubic/axillary hairs of other races; the hairs are distinguished by the multiple irregular intervals of twisting along an otherwise straight hair shaft (Fig. 71-6A).

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FIGURE 71-6 Pili torti. A. Irregularly spaced 180 twists in hair shaft. (From Whiting,15 with permission.) B. Brittle broken hair typical of congenital pili torti. As with trichorrhexis nodosa, pili torti does not signify a particular abnormality but can be seen in many different syndro mes and in the presence of other hair shaft abnormalities. Hereditary pili torti as a n isolated finding, usually autoso mal dominant, but potentially autosomal recessive or sporadic, is present at birth or develops over the first 2 years of life (Fig. 71-6B). Clinically, the patient may have patchy alopecia with coarse stubble or longer broken hairs. The hair abnormality may improve after puberty. Pili torti, or a facsimile best characterized as twisting (Table hair 71-3).

dystrophy, 23

may occur with

other abnormalities

Particularly notable is the association of pili torti with Menkes' syndrome or trichopoliodystrophy. Infants with Menkes' syndrome develop sparse, depigmented brittle hairs that show pili torti or trichorrhexis nodosa on microscopic examination.15 The affected child characteristically has pale, lax skin, and mental and neurologic impairment secondary to degeneratio n of cerebral, cerebellar, and connective tissue.24 In this X-linked recessive disorder, the defective gene, MKN or ATP7A, which maps to Xq13.3, encodes a copper-translocating membrane protein ATPase that prevents effective copper transport and leads to the accumulation of intracellular copper in some tissues.19 The excessive intracellular copper

inappropriately triggers the synthesis of metallothionein, whos e normal function is to chelate copper to prevent cellular toxicity. This further

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deprives the copper-requiring enzymes of the copper needed for norma l function.25 A low serum level of ceruloplasmin is diagnostic. Copper replacement is ineffective in preve nting the inevitable progressive and lethal neurologic decline, but copper-histidine given immediately

postpartum may prevent or ameliorate the severe neurodegeneration. 26

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TABLE 71-3 Causes of Telogen Effluvium TRICHORRHEXIS INVAGINATA

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Trichorrhexis invaginata (bamboo hair ) is a distinctive hair shaft abnormality that may occur sporadically, either in normal hair or with other hair shaft abnormalities, or regularly as a marker for Netherton's syndrome. The primary defect appears to be abnormal keratinization of the hair shaft in the keratogenous zone, allowing intussusception of the fully kera tinized and hard distal shaft into the incompletely keratinized and soft proxima l portion of the shaft.27 This leads to the typical ball-and-socket deformity (Fig. 71-7) or, if fracture of the shaft occurs, a golf tee-shaped distal end of the shaft.

FIGURE 71-7 Trichorrhexis invaginata (light micrograph 400). (From Whiting,15 with permission.) Netherton's syndrome (see Chap. 51) is an autosomal recessive inherited disorder consisting of a triad of ichthyosis, atopic diathesis, and

trichorrhexis invaginata.15 Affected hairs are generally short and

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P.639

brittle and may be irregularly distributed over the scalp; this may lead to potential sampling errors on hair shaft evaluation. The ichthyosis is usually polycyclic ichthyosis linearis circumflexa but may be la mellar ichthyosis or even ichthyosis vulgaris or X-linked ichthyosis. The atopic diathesis may include erythroderma. Recurrent infections, short stature, and mental retardation are rarely reported with Netherton's syndrome. The Netherton's syndrome gene product is LEKTI, a serine protease inhibitor; t he gene (SPINK5) locus is chromosome 5q32. 28 Retinoids and phototherapy may be of value, although the condition may improve spontaneously over time. MONILETHRIX The hair abnormality of monilethrix is distinctive, with extre mely short, brittle hairs emerging fro m keratotic follicular papules29 (Fig. 71-8A). The onset may be delayed until patients are in their teens, and the loss may be localized or diffuse. Microscopically, hairs show elliptical nodes with a regular periodicity of 0.7 to 1 mm.15 Between the nodes, the hair shaft is constricted, and it is in these areas that the hair usually fractures ( Fig 718B). The disorder is caused by mutations in the genes for type II hair keratins hHb1 or hHb6 in the type II keratin gene cluster on chro mosome 12q13.30,31 Both hHb1 and hHb6 are expressed in the hair cortex cells with the abnormality in the helix termination or initiation motifs. 30

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FIGURE 71-8 Monilethrix. A. Keratotic papules in areas of alopecia. B. Typical beaded appearance of hair as seen under light microscopy (40). (From Whiting,15 with permission.) Most cases of monilethrix are of autosomal do minant inheritance, with variable expressivity the hair defect can be mild and localized to the occiput.15,31 The hair defect may occur alone or in association with

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keratosis pilaris, physical retardation, syndactyly, cataracts, and nail/teeth abnormalities. Retinoids and topical minoxidil may be useful treat ments, although this condition may also improve spontaneously with age. Hair shaft abnormalities associated with unruly hair UNCOMBABLE HAIR SYNDROME The distinctive hair of patients with uncombable hair syndrome ma y present any time from infancy to puberty. The slow-growing, silvery blonde, spun-glass hair is generally unmanageable and disorderly

but not unduly fragile15,32 (Fig. 71-9A). The condition may be autosomal dominant or sporadic. Under light microscopy, the hair appears normal or may have some suggestion of a longitudinal groove or ribbon-like flattening (Fig. 71-9B). Scanning electron microscopy confirms a

longitudinal groove, and the hair may, if viewed on cross -section, show a kidney bean or triangular shape, which accounts for the alternate ter m of pili trianguli et canaliculi. The longitudinal groove in the hair shaft is not specific to this syndrome but can be seen in normal hair, in other etiologies of unruly hair, and in several types of ectodermal dysplasia. 15 The defect may be secondary to an abnormal configuration of the inner root sheath, which keratinizes before the hair shaft and thus determines its shape. 32 Biotin supplementation has been advocated in one case report,33 but generally there is no effective treatment for the syndro me.

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FIGURE 71-9 Uncombable hair syndrome. A. Typical clinical picture.

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(Photograph courtesy of Vera H. Price, MD.) B. Longitudinal groove seen in uncombable hair syndrome (light micrograph 400). (From Whiting,15 with permission.) WOOLY HAIR Wooly hair is the presence of Negroid hair on the scalp of persons of nonNegroid background. Microscopically, the hairs are tightly coiled. The unruly hair presents at birth or in infancy, usually as a solitary proble m inherited in an autosomal dominant fashion.34 However, two families wit h either autosomal dominant or autosomal recessive inheritance and

associated palmoplantar keratoderma and cardiac abnormalities have been reported.35 A sporadic variant has been reported with fine white-blond hair (Fig. 71-10). Diffuse partial wooly hair, a recently described autosoma l dominant condition, presents in young adulthood with two distinct populations of scalp hair, one straight and the other ver y curly.36 The curly hairs are thinner than normal hairs, which may contribute to the clinical appearance of a reduction in hair density.

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FIGURE 71-10 Sporadic recessive wooly hair. MARIA-UNNA TYPE OF HEREDITARY HYPOTRICHOSIS This autosomal dominant condition is unusual in that the hair abnormalit y varies with age. The scalp hair in Maria-Unna type of hereditary hypotrichosis is sparse or absent at birth, with variable coarse, wiry hair regrowth in childhood, and potential loss again at puberty. 37 Body hair is sparse to absent. Light microscopic examination of the hairs shows irregular twisting, and scanning electron microscopic examination shows longitudinal ridging and cuticle peeling. Diffuse follicular hyperkeratosis and facial milia-like lesions may be present. A distinct gene in

chro mosomal region 8p21 close to the hairless (hr) locus has been noted.38 P.640

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Hair shaft abnormalities unassociated with breakage or unruly hair There are a few specific abnormalities of the shaft that should be noted here because of their frequency. The hair in pili annulati, an autosoma l dominant (occasionally sporadic) condition, shows alter nating light and dark bands both clinically and under light microscopy secondary to airfilled spaces in the cortex.39 Cuticle folding and holes in the cortex are seen with electron microscopy.15 Clinically, the hair in pseudo pili annulati shows a similar light microscopic picture, but the condition is nonhereditary and is not associated with any hair shaft corte x

abnormality.40 The cause of the latter appears to be periodic flattening of the hair shaft that causes light to be reflected as bright bands. Both conditions appear primarily in blond hair and are not associated with hair breakage. With transverse illumination, the banding in pili annulati is seen in whatever way light strikes the hair, whereas pseudo pili annulati is seen only when the hair is rotated into certain positions.39 ABNORMALITIES OF HAIR CYCLING Telogen effluvium This common type of hair loss may occur at any age and represents a precipitous shift of a percentage of anagen hairs to telogen. Telogen effluvium is a reaction pattern to a variety of physical or mental stressors; the most common causative factors are given in Table 71-3. Any drug can potentially cause telogen effluvium, although some classes of drugs routinely cause this.

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The clinical increase in scalp hair shedding (over a normal of 50 to 100 hairs per day) usually begins as the first group of anagen hairs to be thrown prematurely into telogen finally completes telogen and moves once again into anagen, 3 to 4 months after the inciting event. If the inciting cause is removed the shedding will resolve over the next several months as the percentage of hairs in telogen return to normal; however, the hair densit y may take 6 to 12 months to return to baseline. In a significant number of patients, no obvious cause is found for teloge n effluvium, and the increased shedding, and concomitantly the decreased density, of the scalp hair may become chronic.41 Telogen effluvium is always potentially reversible and does not lead to total scalp hair loss, as the percentage of hairs in telogen rarely goes beyond 50 percent (Fig. 71-11).

FIGURE 71-11 Telogen effluvium. Anagen effluvium

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The daily loss of some telogen hairs is entirely normal, but it is always abnormal to shed anagen hairs. The term anagen effluvium, as currently used to describe the pathologic loss of anagen hairs, is somewhat misleading because the abnormal anagen hairs in this condition are usually broken off rather than shed. The anagen hairs in loose anagen syndrome, however, are shed in toto. The classic and easily recognizable causes of anagen effluvium of the scalp are radiation therapy to the head and systemic chemotherapy, P.641

especially with alkylating agents. These agents can impair or totally disrupt the anagen cycle. The net result is either anagen hairs that break off within the follicle or at the level of the scalp secondary to a weak point in the hair shaft and are then shed without roots, or dystrophic anagen hairs that are easily dislodged from the usual follicular moorings. Replacement with a normal pelage usually occurs rapidly after discontinuation of chemotherapy although high-dose busulfan as part of the preparatory treatment for bone marrow transplantation may lead to permanent alopecia.42 Regrowth after radiation therapy depends on type, depth, and dose-fractionation.43 In the absence of these obvious causes of anagen disruption, one must consider exposure to toxic agents. Mercury intoxication, through either chronic industrial exposure, consumption of polluted water or seafood, or inadvertent exposure to mercury-containing antiseptics or fungicides, can lead to hair loss with or without other symptoms, especially neurologic ones.44 Diagnosis is by documenting elevated mercury levels in hair, blood, or urine. Boric acid intoxication may be through exposure to

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household pesticides or ingestion of some common ho usehold products in which boric acid is a preservative. Patients may develop hair loss along with gastrointestinal, central nervous system, and renal symptoms, a hemorrhagic diathesis, and exfoliation or bullae.43,45 Blood boric acid levels are elevated in affected patients. Thallium poisoning has the most dramatic associated hair loss, wit h complete epilation occurring 2 to 3 weeks after intoxication. 46 Acutely, patients experience primarily neurologic symptoms including irritability, dysesthesia, ataxia, convulsions and coma. Blood and urine levels are positive at the time of acute poisoning. Toxic exposure to colchicine and ingestion of certain plants can lead to anagen loss.43 Severe protein malnutrition may also lead to anagen effluvium. Arsenic exposure does not cause hair loss; instead, arsenic is concentrated in the hair, which facilitates a diagnosis long after

intoxication may have occurred. Loose anagen syndrome This recently described syndrome has been primarily described in fairhaired children who have easily dislodgable hair (Fig. 71-12A).47,48 However, loose anagen syndrome can occur in adults and may be familial. This condition can be, and probably previously was, misdiagnosed as telogen effluvium if the shed hair roots are not exa mined micros copically. The hair loss may be inapparent except when traumatic pulling or extraction easily dislodges clumps of hair; this is particularly so in the parents of affected children, who themselves may have a mild, but previously unrecognized, variant of the condition that clinically may appear indistinguishable fro m a mild persistent telogen effluvium. The hair may

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also

present

with

unruliness,

occasionally

causing

confusion

with

unco mbable hair syndrome or wooly hair (Fig. 71-12B). Microscopically, the proximal shed hair shows a normal anagen bulb, with a ruffled cuticle but without the usual attached root sheath7 (see Fig. 71-2B). A scalp biopsy, which is unnecessary for diagnosis, may show premature and abnormal keratinization of the inner root sheath, wit h clefts between the inner and outer sheath and the hair shaft. The hair loss may improve with age, and gentle handling decreases shedding.

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FIGURE 71-12 Loose anagen syndrome. A. Diffuse hair loss. B. Unruly hair. (From Olsen,7 with permission.) Alopecia areata At any given time, approximately 0.2 percent of the population has alopecia areata and approximately 1.7 percent of the population will experience an episode of alopecia areata during their lifetime.49,50 Clinically, patients with alopecia areata may have patchy or confluent hair loss on the scalp and/or body (Fig. 71-13A). Alopecia totalis refers to the total absence of terminal scalp hair, and alopecia universalis refers to the total loss of terminal body and scalp hair.51 Ophiasis refers to a bandlike pattern of hair loss over the periphery of the scalp (Fig 71-13B). Hair loss may also be diffuse, mimicking anagen effluvium (Fig. 71-13C).

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FIGURE 71-13 A. Extensive patchy alopecia areata. B. Ophiasis pattern of alopecia areata. C. Diffuse pattern of loss in alopecia areata. (Reprinted with permission from Hardinsky MK: Alopecia areata, in Disorders of Hair Growth: Diagnosis and Treatment, edited by EA Olsen. New York, McGrawHill, 1994.) The scalp appears normal in alopecia areata. In affected areas, anagen is abruptly terminated prematurely and affected hairs move prematurely into telogen, with resultant often precipitous point hair hairs shedding. may be The near

pathognomonic

exclamation

present,

particularly at the periphery of areas of hair loss (Fig. 71-14). These short broken hairs, whose distal ends are broader than the proximal ends, illustrate their inherent sequence of events: follicular damage in anagen and then a rapid transformation to telogen. White or graying hairs are frequently spared and probably account, in cases of fulminant alopecia areata, for the mysterious pheno menon of going gray overnight. There is an increased incidence of autoimmune diseases in patients with alopecia areata, particularly thyroid-related disease,52 and there is a higher prevalence of pigmentary defects in patients with alopecia areata. 53 Nails in patients with alopecia areata may show fine pitting or, less commonly, mottled lunula, trachyonychia, or onycho madesis.54

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FIGURE 71-14 Excla mation mark hairs of alopecia areata. (From Olsen EA: Clinical tools for assessing hair loss, in Disorders of Hair Growth: Diagnosis and Treatment, edited by EA Olsen, New York, McGraw-Hill, 1994, with permission.) A scalp biopsy is generally unnecessary to establish the diagnosis of alopecia areata, except in the uncommon presentation of diffuse shedding in which telogen or anagen effluvium is also a consideration. Typically, a biopsy of involved scalp shows a peribulbar, perivascular, and outer root sheath mononuclear cell infiltrate of T cells and macrophages.55 Follicular dystrophy, including abnormal pigmentation and matrix degeneration, may also be present.56

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The pathogenesis of alopecia areata is still obscure, although most authors tend to classify alopecia areata as an autoimmune disease. As opposed to normal hairs, strong major histoco mpatibility complex (MHC) P.642

class I and class II immunoreactivity are found in lesional alopecia areata follicles, which also display aberrant expression of adhesion molecules known to direct hematopoietic cell migration.54 One potential explanation for the failure of repression of MHC expression necessary for autoimmunity to develop in alopecia areata is the release of cytokines by certain stimuli, including trauma, neurogenic inflammation, or infectious agents. The elusive follicular autoantigens so exposed may be of keratinocyte or melanocyte origin, but the almost exclusive attack on melanogenically active anagen follicles makes this a particularly attractive hypothesis. That there is a genetic association with both susceptibility to and severity of alopecia areata is clearly shown by recent human leukocyte antigen (HLA) studies.57 Spontaneous re mission is common in patchy alopecia areata, but is less so with alopecia totalis or universalis.52 Spontaneous or treatment-related regrowth is also adversely affected by the location of hair loss (ophiasis pattern is particularly recalcitrant), the age of onset (children younger than 5 years old with alopecia totalis or universalis have the worse prognosis), association of atopy, and duration of hair loss in a given area. 52,58 Current treatment is not, at this point, directed at the etiology of alopecia areata but rather at the resulting inflammatory infiltrate and (presumably) the growth

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inhibitory factors produced by this response. Relapse is common, both acutely and over a lifetime. Treatment of alopecia areata is with either immunosuppressives (local or systemic) or with irritants/immunogens, and is generally tailored to the severity of the disease.54 For localized patchy alopecia areata, intralesional steroids given at 4- to 6-week intervals are usually effective, with the main side effect being local dermal/subcutaneous atrophy related to the depth and concentration of injected steroid. Topical glucocorticoids classes I to V are also effective but take several months for initiation of hair growth, rather than the weeks for intralesional steroids. Side effects of topical steroids are generally limited to acne/hypertrichosis on the face fro m inadvertent transfer from the scalp and local epidermal atrophy with the more potent steroids. Systemic steroids, particularly short courses (less than 8 weeks) of tapering doses, are often used either alone or in conjunction with topical agents. In this setting, acne and weight gain are commonly seen side effects.59 PUVA is another immunosuppressant treatment that may be effective in alopecia areata, particularly in patients with extensive scalp and body hair loss. Between 30 and 80 treatments may be necessary before hair induction occurs, and there is an increased risk of photodamage/photoaging and skin cancer with PUVA use.60 P.643

The immunostimulation of topical irritants, especially anthralin, or topical immunogens (diphencyprone, squaric acid dibutylester) can be very effective in alopecia areata, but their use runs the risk of intolerable

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irritation if the dose titration is inappropriate. The particular mechanism of action of contact dermatitis that makes it a treatment for alopecia areata is purely speculative at this time, but it may include enhanced clearance of the putative follicular antigens through recruit ment of new T cells and antigenic competition and interference with the initial or continued production of proinflammatory cytokines by the follicular keratinocytes. 61 Five percent topical minoxidil, as a nonspecific hair growth promoter, may be a useful drug as a single agent or as an adjuvant with topical anthralin.62 There is a low incidence of local dryness/irritation and facial hypertrichosis with topical minoxidil. Although rare, the potential for systemic effects of topical minoxidil, particularly in young children, must be considered and the total amount applied kept to the recommended 2 mL/day. Patients with alopecia areata need psychological support and physical means of camouflaging their hair loss. The latter often requires the use of a wig, which should be considered an integral part of treat ment in patients with extensive scalp hair loss. The National Alopecia Areata Foundation is an excellent source of information for patients (www.naaf.org). Focal Hair Loss NONSCARRING HAIR LOSS Production decline TRIANGULAR (TEMPORAL) ALOPECIA Triangular alopecia may be congenital but usually appears in childhood as a focal patch of hair loss. The hair loss either may be complete or fine vellus hairs may remain.13,63 The underlying scalp is normal. The temporal region is a common location, and the hair loss is frequently

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bilateral (Fig. 71-15). Histologically, the affected area shows a transitio n from terminal to vellus hair. The alopecia is usually persistent.

FIGURE 71-15 Triangular alopecia. PATTERN HAIR LOSS The term androgenetic alopecia was previously applied to both men and women with a very common, potentially reversible scalp hair loss that generally spares the Hippocratic wreath portion of the scalp. The

hair loss condition in men and women has in common miniaturization and shortening of anagen duration of affected hairs and, consequently, an increased percentage of affected hairs in telogen.64,65 Men with this type of alopecia tend to have somewhat synchronous behavior of hairs in the four different regions of the top of the scalp (vertex, mid, frontal, and bite mporal) along with potentially profound degrees of miniaturization leading to recognizable patterns of hair loss (Fig. 71-16).65 In men, this

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male pattern baldness is hereditary (probably autosomal do minant) and androgen dependent, specifically dihydrotestosterone-related.64,66

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FIGURE 71-16 Hamilton-Norwood classification of pattern hair loss in men. (From Olsen,65 with permission.) Women, on the other hand, have lesser degrees of miniaturization of terminal hairs in affected areas and, hence, rarely any balding when

compared to men. Patterning is less obvious then in men, although three patterns of hair loss do exist in women (Fig. 71-17A, Fig. 71-17B).65 Not all women with pattern hair loss ha ve proven androgen-dependence. Wome n with profound hyperandrogene mia, which is usually tumor related, ma y develop a Hamilton pattern of hair loss or severe diffuse central scalp hair loss, and women with other stigmata of

hyperandrogenemia/hypersensitivity, such as those with polycystic ovarian syndrome, may present with pattern hair loss in the second to third decades.65 The majority of women with pattern hair loss, however, have no increase in serum androgens, no other signs/sympto ms of androgen hypersensitivity, and do not respond to androgen inhibition with reversal of hair loss.64,65 Therefore, the preferred more encompassing

umbrella

term for this hair loss in women is female pattern hair

loss. Subcategories of early onset female pattern hair loss with or without androgen excess and late postmenopausal onset fe male

pattern hair loss with or without androgen excess 65 will allow sorting out of the genetic (e.g., polycystic P.644

ovarian syndro me and early male pattern baldness appear genetically linked)67 and cellular mechanis ms of these various subtypes.

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FIGURE 71-17 Pattern hair loss in women. A. Different phenotypic expressions. B. Characteristic frontal accentuation. Androgen-mediated hair growth (or loss) requires formation of an

androgen-androgen receptor complex, which then binds to the androgenresponse element DNA-binding site leading, in turn, to transcription of

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certain protein(s).64 Androgens are 19-carbon steroid structures (Fig. 7118), that are normally produced by both adrenal glands and gonads. The most potent androgens (testosterone, dihydrotestosterone, and

androstenediol) are those with a 17-hydroxy group, as this moiety is necessary for high-affinity androgen receptor binding. The weaker 17ketosteroids, such as dehydroepiandrosterone and androstenedione, assume importance by their interconversion by 17-OH steroid dehydrogenase to more potent androgens at the end-organ site, including the hair follicle. The enzyme 5-reductase converts testosterone to dihydrotestosterone, whic h has greater affinity and avidity for the androgen receptor.68 Two isozymes of 5-reductase, called 5R1 and 5R2, have been cloned and their corresponding genes are located on chro mosomes 5 and 2 respectively. 66 Although type I 5-reductase appears to be more ubiquitously distributed in skin, particularly in the sebaceous gland, 5-reductase type 2 is found in the outer root sheath69 and dermal papillae of hair follicles, but is differentially expressed in various tissues.64 That 5-reductase type 2 is involved in male pattern baldness is suggested by the absence of balding in men with 5-reductase type 2 deficiency;66 the increased expression of 5-reductase in balding versus nonbalding scalp;70 the results in animal models of androgenetic alopecia showing reversal of hair loss with type 2 but not type 1 5-reductase inhibitors;64 and the response of men wit h male pattern baldness to finasteride, an inhibitor of 5-reductase type 2.71

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FIGURE 71-18 Androgen pathway. (From Kaufman,66 with permission.) Effective treatment of pattern hair loss in both men and women can include both medical and surgical approaches. Topical minoxidil (2% and 5%) is a nonspecific hair-growth pro moter affecting anagen induction, duration, and size of hair shaft. Although the mechanism of action in hair growth promotion is unclear, its calcium channel opener activity appears to be important.64 The medication should be applied to the scalp twice a day, with the earliest clinical response seen at 4 to 6 months and generally a maximum response at 1 year.64 About 20 to 25 percent of persons so treated will have notable clinical regrowth, although most patients will experience at least a stabilization of loss.72 There is slight risk of facial hypertrichosis and of irritation/allergic contact dermatitis, secondary to

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either the minoxidil or propylene glycol. Both side effects are more frequent with the 5% versus the 2% preparation. 64 Whether the facial hypertrichosis is from inadvertent transfer from the scalp or secondary to a local reaction to low levels of serum minoxidil is not clear. Surgical treat ment of pattern hair loss has undergone dramatic improve ment in recent years73 (see Chap. 277). It is based on the premise of donor dominance whereby hairs from a nonandrogen-dependent site (occiput)

can be successfully transplanted to a bald andro gen-dependent site. Cosmetic coverage is currently limited by the amount and density of available occipital donor hair and the expertise of the surgeon. Ideally, male candidates for this procedure should be those in whom final resculpturing of the frontal hair line has naturally occurred. A combinatio n of minigrafts (1.5- to 2.5-mm grafts) and micrografts (one to two hairs each graft) of donor hair are used more frequently now than the once standard 4 mm plugs to fill in areas of baldness. The micrografts are particularly useful because they do not require removal of a plug of tissue into which to insert the graft; rather, a small hole or incision can be made to accommodate a single or a few donor hairs. Micrografting is the surgical treatment of choice in wo men with pattern hair loss, who, unlike men, never develop baldness and for whom the use of standard hair transplants means sacrificing terminal hair when a recipient plug of tissue is removed. For wo men with pattern hair loss, at least those who have doc umented androgen excess or androgen hypersensitivity, the use of medications that block either the production of or the cellular utilization of androgens may be helpful. Although the systemic antiandrogens spironolactone (in doses 100 mg daily),74 flutamide (in doses of 250 to 500 mg bid to tid),75

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and cyproterone acetate76 have shown some effectiveness in women with pattern hair loss, none of these have been studied in large placebocontrolled trials or with stratification of women by presence or absence of hyperandrogenism. This is especially important today because there is a lower threshold, given our knowledge of coincident insulin resistance, to identify polycystic ovarian syndrome, a potential cause of

hyperandrogenism and concomitant pattern hair loss. Spironolactone is a potassium-sparing diuretic whose main side effects are hyperkalemia, irregular menses, and breast tenderness/bloating. Flutamide users must be monitored for potential P.645

hepatotoxicity. Because both drugs can cause feminizat ion of a male fetus, they should be used only in women of non-childbearing potential or in those wo men of childbearing potential who are using effective

contraception, preferably combination oral contraceptive pills. Cyproterone acetate (CPA) is marketed outside the United States in conjunction wit h ethinyl estradiol, either in a reversed sequential regimen of 100 mg CPA on days 5 to 15 and 50 g ethinyl estradiol on days 5 to 25, or in a low-dose combined formulation of 2 mg CPA and 50 g ethinyl estradiol on days 5 to 25. Side effects are similar to those of oral contraceptive pills.64 Topical antiandrogens, which theoretically could also be used in men, are not commercially available at this time. Men with androgenetic alopecia may use a systemic 5 -reductase inhibitor without the biologic concerns of emasculinization seen with systemic antiandrogens since there is neither a decrease in testosterone levels nor

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any effect on androgen-receptor binding. In men with androgenetic alopecia, placebo- controlled studies of the type 2 5-reductase inhibitor finasteride have shown increased hair growth in ~50 percent of men at 1 year and 66 percent by 2 years.71 The placebo-treated group, by comparison, had hair growth in 7 percent at 1 year and a progressive decline at 2 years that continued over a 5-year follow-up.77 Sexual adverse events (1.8 percent of those on 1 mg finasteride versus 1.3 percent of those on placebo) were the only significant side effects and generally cleared, either on or off treatment.71 Hair breakage TRICHOTILLOMANIA Trichotillomania (Greek, hair pulling madness ) is a co mmon, but difficult to manage, cause of focal scalp hair loss. It is classified as an impulse control disorder in which patients pull, pluck, or cut their hair.78,79 The clinical presentation is usually quite distinctive, with a confluence of very short sparse hairs within an otherwise normal area of the scalp (Fig. 71-19A, Fig. 71-19B). Microscopic exa mination of the ends of cut or plucked hairs generally reveals either the tapered tips of newly regrowing anagen hairs or bluntly cut hairs. (A hair pull here is usually negative because the telogen hairs have generally all been dislodged) The differential diagnosis includes alopecia areata and tinea capitis, and because patients generally deny any role in the hair loss, these usually need to be definitively ruled out. A scalp biopsy can be diagnostic, showing the characteristic increase in the number of catagen hairs (rarely seen in biopsies of normal scalp), trichomalacia, and melanin within the follicular

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canal secondary to traumatic hair removal and the absence or sparsity of a perifollicular inflammatory infiltrate.80,81

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FIGURE 71-19 Trichotillomania. A. Bizarre pattern of localized hair loss in a boy. B. Extensive hair loss in a woman. Treatment of these patients is challenging. Children with trichotillomania may have a form of habit tic, which can be broken by mere

acknowledgment of the problem or behavior modification. Adolescents and adults with this condition, who tend to be primarily fe males, are usually particularly psychological reluctant to accept and/or the diagnosis to and help often require their

intervention

medication

modify

behavior.78,79 Clomipramine may be particularly effective. TRACTION ALOPECIA Traction alopecia is caused by inadvertent prolonged traction on the scalp by the physical pressure of tight braids, certain hair styles (e.g., pony tail), foa m rollers, etc (Fig. 71-20). While potentially reversible, the hair loss may be persistent if the traction is unrelenting over months to years.

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FIGURE 71-20 Traction alopecia. TINEA CAPITIS (See also Chap. 205) Tinea capitis is a very common cause of hair breakage or loss, particularly in children. Typically, there is either a seborrheic dermatitis presentation, with or without erythema of the scalp (Fig. 71-21A), or a noninflammatory black dot ring-worm presentation, with broken hairs filling, but not

projecting from, follicular orifices (Fig. 71-21B). Less commonly, tinea capitis can present as a pyoderma-like kerion.

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FIGURE

71-21

Tinea

capitis.

A.

Endothrix

infection

caused

by

Trichophyton tonsurans presenting as seborrheic dermatitis. B. Endothrix infection caused by T. tonsurans. Note black dots of broken,

infected hairs within the follicular canals. (Reprinted with permission from DeVillez RL: Infections, physical, and inflammatory causes of hair and scaly abnormalities, in Disorders of Hair Growth: Diagnosis and

Treatment, edited by EA Olsen. New York, McGraw-Hill, 1994, pp 7190.) The etiologic agent(s) of tinea capitis varies in different parts of the world. Currently in the United States, in which the condition is far more prevalent in African Americans and Hispanics than in Caucasians, the usual fungal isolate is Trichophyton tonsurans.82 This is an endothrix infection and KOH examination of affected hairs shows arthrospores and hyphae interspersed among the keratin fibers of the hair shaft. To establish a diagnosis, hairs should be cultured as well as examined after KO H preparation, since a positive yield by KOH alone is dependent on the amount of inflammation and may vary fro m 29 to 66 percent. 83 Only ectothrix fungal infections fluoresce under Wood's light. Treatment of tinea capitis must be by the systemic route, and contacts must be sought and treated to prevent reinfection. Asymptomatic carriers are common. Treatment is with griseofulvin, terbinafine, or one of the newer azoles.82,84,85 Griseofulvin, 20 to 25 mg/kg per day of the microsiz ed product (or 10 to 15 mg/kg of the ultramicrosized product), is given with a fat-containing meal until the culture is negative (generally 6 to 10 weeks).82 Griseofulvin is fungistatic. Terbinafine, an allylamine, is not

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significantly affected by food and is fungicidal: doses of 62.5 to 250 mg qd for 4 weeks (62.5 mg/day for those weighing <20 kg, 125 mg/day for those weighing 20 to 40 kg, 250 mg/day for those weighing > 40 kg) are effective. The azoles, itraconazole at 100 mg qd, or fluconazole 6 to 8 mg/kg for 4 to 6 weeks are effective alternative treatments. Both latter drugs inhibit the cytochrome P 4 5 0 system so drugdrug interactions should be considered. Fluconazole P.646

bioavailability is not affected by food. Topical sporicidal agents, such as selenium sulfide or ketoconazole, help to limit the spread of infectious spores. Primary or acquired localized hair shaft abnormalities ACQUIRED LOCALIZED TRICHORRHEXIS NODOSA Acquired localized trichorrhexis nodosa may be seen in hair that is subject to repetitive rubbing, such as with lichen simplex chronicus or

trichotillomania. It may also present in focal areas secondary to trauma from chemical or P.647

heat processing of hair. Acquired pili torti may present as a focal patch of fragile hair. This is usually secondary to trauma or some underlying, potentially scarring, scalp abnormality.15 Hypervita minosis in patients with anorexia nervosa or retinoids have also been associated with acquired pili torti. Bubble hair is a recently recognized and very distinctive abnormality of the hair shaft and is characterized by rows of bubbles seen

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microscopically within localized areas of brittle hair.86 Exposure to prolonged high temperatures fro m curling irons or hair dryers are the usual causative factors, and the defect is completely reversible. Unruly hair WOOLY HAIR NEVUS Wooly hair nevus is a nonhereditary focal condition which usually appears within the first 2 years of life but can occur as late as adolescence. 87 Fifty percent of cases have an associated epidermal, verrucous, or pigmented nevus, although not necessarily immediately under the affected hair. 15 There may be associated ocular abnormalities, such as persistent papillary membrane or retinal abnormality. Spontaneous improvement in the hair may occur with age. ACQUIRED PROGRESSIVE KINKING This condition has been primarily reported in postpubescent males with androgenetic alopecia.88 It presents with gradual curling and darkening of the frontal, temporal, auricular, and vertex hairs. Microscopically, affected hairs show kinks and twists, with or without longitudinal grooving. Abnormality of cycling ALOPECIA AREATA As noted previously, alopecia areata can (and usually does) present as focal hair loss. SYPHILIS Hair loss may be one or the sole cutaneous manifestation of secondary syphilis. This may present as a patchy moth-eaten alopecia or as

generalized thinning (Fig. 71-22). A scalp biopsy may show either a superficial and deep perivascular mixed lymphocytic /macrophage/plasma

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cell infiltrate, a peribulbar perifollicular lymphocytic infiltrate mimicking alopecia areata, or a noninflammatory telogen effluvium picture.89,90 Serologic testing should be positive, and treat ment with appropriate antibiotics will reverse the hair loss.

FIGURE 71-22 Alopecia of secondary syphilis. Scalp conditions associated with focal hair loss Most eczematous conditions of the scalp do not cause hair loss, the exception being pityriasis amiantacea, severe scalp psoriasis and

malignancies such as cutaneous T cell lymphoma or histiocytosis X. In pityriasis amiantacea, thick tenaciously adherent scale infiltrates and surrounds the base of a group of scalp hairs91 (Fig. 71-23). The conditio n may mimic psoriasis clinically, but, in contradistinction to psoriasis, the hair in involved areas is dislodged on atte mpts to physically remove the scale. Removal of the scale in this manner can lead to scarring alopecia.

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The condition usually presents in children, is best treated with keratolytics, and usually improves with age.

FIGURE 71-23 Pityriasis amiantacea. Note area of patchy alopecia after manual re moval of adherent scale. CICATRICIAL ALOPECIA (DESTRUCTION OF THE FOLLICLE) The term cicatricial or scarring alopecia implies the potential of permanent destruction of the hair follicle. Clinically, there is effacement of follicular orifices, always in a patchy or focal distribution. A biopsy is confirmative, showing replacement of follicles with fibrotic stellae and either fibrosis or hyalization of surrounding collagen. 92,93 and 94 Although some cases of cicatricial alopecia are due to physical or develop mental causes (e.g., pressure or aplasia cutis congenita), or to the hair follicle being secondarily involved in a destructive process (e.g.,

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kerion

fungal infection or metastatic/primary neoplasm), most

patients seeking medical attention have a primary cicatricial alopecia. Although these conditions have a common endpoint, they have varied clinical and histologic features and virtually no therapies able to turn the process completely off. There is direct evidence in the mouse, and indirect evidence in the human, that compromising the integrity of the sebaceous gland and/or b ulge is important in the development of the scarring process in the primary cicatricial alopecias.95 Selective destruction of the stem cell region in mice and graft versus host disease,96 in which an inflammatory infiltrate involves the stem cells, can lead to follicular destruction. Moreover, alopecia areata, in which the infla mmation spares the stem cell area, does not lead to permanent hair loss. In the asebia mouse, which lacks one gene responsible for normal sebum production, the hair follicle is destroyed when the shaft is unable to exit the follicle properly. 95 Other animals wit h sebaceous gland pathology also develop cicatricial alopecia. A new classification system based on the type of infla mmatory infiltrate on biopsy (Table 71-4) along with recommended standardization of biopsy site, processing and pathology parameters, and cataloguing of clinical findings,97 will help us to identify and evaluate significant differences between these entities. The primary cicatricial alopecias are presented in keeping with this classification system.

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TABLE 71-4 Proposed Working Classification of Primary Cicatricial Alopecia Primary cicatricial alopecia LUPUS ERYTHEMATOSUS (See also Chap. 171)

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Chronic cutaneous lupus erythematosus may present in the scalp, usually with erythema, P.648

atrophy and variable hypopigmentation and/or follicular plugging ( Fig. 7124). Patients may have no other clinical lesions nor serologic evidence of lupus erythematosus. Scalp biopsy is generally confirmative, showing vacuolar degeneration of the basal cell layer, a perivascular and

periadnexal lymphoid infiltrate, increased dermal mucin and sebaceous gland loss.92 Direct immunofluorescence studies most commonly

demonstrate granular deposits of IgG and C3 at the dermalepidermal junction and at the junction of the dermis and follicular epithelium. Potentially effective treatments include topical, intralesional, and systemic steroids, antimalarials, systemic retinoids, and thalido mide.93,98

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FIGURE 71-24 Lupus erythematosus. LICHEN PLANOPILARIS Areas of active alopecia in lichen planopilaris are clinically distinguished by perifollicular erythema and/or a violaceous discoloration of the scalp (Fig. 71-25). Keratotic follicular papules may be evident. Evidence of lichen planus may be present elsewhere, and this should be sought to help confirm the diagnosis. Histologically, there is a perifollicular lymphoid, often bandlike, infiltrate primarily in the infundibular and isthmus portions of the follicle, with or without the presence of adjacent colloid bodies. The typical overlying histologic changes of lichen planus may or may not be present; these include and sawtooth rete ridges, interface dermatitis, findings

hypergranulosis,

Civatte

bodies.99

Immunofluorescence

consist of globular deposits of IgM adjacent to follicular epithelium and

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patchy or linear fibrogen deposits along the basement membrane zone. Glucocorticoids are the mainstay of treatment.

FIGURE 71-25 Lichen planopilaris. Lichen planopilaris histologically can be seen in two other disparate conditions. Graham-Little syndrome is characterized by lichen planus -like lesions and a follicular spines /keratosis pilaris-like picture that develop in areas of alopecia on the scalp, eyebrows, axillary, and pubic areas.92 Frontal fibrosing alopecia is a term given to the frontote mporal hairline recession and eyebrow loss in postmenopausal women that is associated with perifollicular erythema, especially along the hairline. 100 Scalp biopsy is indistinguishable from lichen planopilaris. Topical and intralesional steroids, topical and systemic retinoids, and hormone

replacement therapy do not prevent the hair loss progression; oral steroids

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and chloroquine have been demonstrated to slow the progression in a few patients. A lichenoid inflammatory infiltrate has also been seen in a progressive inflammatory (perifollicular erythema and follicular keratosis) scarring alopecia limited to the area of pattern hair loss.101 There appears to have some overlap with frontal fibrosing alopecia. PSEUDOPELADE OF BROCQ In clinical terms, pseudopelade of Brocq implies flesh- to pink-colored, irregularly shaped alopecia that may begin in a moth-eaten pattern with eventual coalescence into larger patches of alopecia102 (Fig. 71-26). There has been considerable debate as to the specificity of this diagnosis versus an assignation of the term to describe all noninflammatory scarring alopecias, including the end-stage of a variety of initially inflammatory conditions. Histologically, the lesions are characterized by a perifollicular and perivascular lymphocytic infiltrate primarily at the level of the follicular infundibulum, loss of sebaceous epithelium, and fibrotic streams into the subcutis without interface or follicular plugging changes. 103 Elastin stains may distinguish pseudopelade (persistent elastic fibers around the midshaft of the follicle) from lichen planopilaris and lupus erythematosus (loss of P.649

elastic fibers in this location).104 Direct immunofluorescence is negative in the majority of cases. It is unclear what treatment specifically helps.

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FIGURE 71-26 Pseudopelade of Brocq. CENTRAL CENTRIFUGAL CICATRICIAL ALOPECIA Recently, a subset of cicatricial alopecia has been identified in African Americans and given the name of central centrifugal cicatricial alopecia (CCCA).97 This condition has also been called hot co mb alopecia, follicular degeneration syndrome, and central centrifugal scarring alopecia, the latter meant to be an umbrella term for follicular degeneration syndrome as well as other causes of central scalp hair loss. 105 Affected patients with CCCA show follicular loss primarily over the crown, with little in the way of either bogginess or tautness to the scalp ( Fig. 71-27). Inflammation has been reported more commonly in affected men than in affected women.106 Histologically, the earliest and most distinctive change is premature desquamation of the inner root sheath with later changes

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including migration of the hair shaft through the outer root sheath, a mononuclear infiltrate primarily at the isthmus, and, finally, loss of the follicular epithelium and replacement with fibrous tissue. 106 Although tight braiding, hot combs, and hair straightening agents are often invoked as causative or at least contributory factors and their use discouraged in CCCA, this has not been definitely proven. The distribution of CCCA overlaps that of pattern hair loss but the sex distribution is tremendously skewed toward women.

FIGURE 71-27 Central centrifugal cicatricial alopecia. ALOPECIA MUCINOSA Alopecia mucinosa generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp and face. 94 Biopsy reveals prominent follicular, epithelial and sebaceous gland mucin, and perifollicular lymphohistiocytic infiltrate without concentric la mellar fibrosis.92,94

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KERATOSIS FOLLICULARIS SPINULOSA DECALVANS Keratosis follicularis spinulosa decalvans (KFSD) is an X -linked disorder with the frequently gene located at Xp22.13p22.2.107 Female carriers are The disorder is characterized by follicular

affected.

hyperkeratosis, scarring alopecia of the scalp, absence of eyebrows and sometimes eyelashes, severe photophobia, and resulting corneal dystrophy. Onset is in early childhood and the symptoms decrease with age. Histopathologically, there is plugging o f the pilosebaceous orifices with keratinaceous debris, and superficial and deep perivascular and

periappendageal infiltrate of lymphocytes and plasma cells. Retinoids may be useful in treatment. FOLLICULITIS DECALVANS Folliculitis decalvans is an inflammatory alopecia that leads to bogginess or induration of involved parts of the scalp along with pustules, erosions, crusts, and scale108 (Fig. 71-28). Predictably, Staphylococcus aureus is usually cultured from these pustules, but whether this is a primary or secondary process is unclear. Histologically, early lesions show an acute suppurative folliculitis with neutrophils and eosinophils, later mixed with lymphocytes and histocytes.92,93 Loss of sebaceous epithelium and perifollicular fibrosis is common.92 Systemic antibiotics with or without rifampin, systemic and/or topical steroids, and systemic retinoids may also be helpful.108

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FIGURE 71-28 Folliculitis decalvans. DISSECTING FOLLICULITIS Dissecting folliculitis or perifolliculitis capitis abscedens et suffodiens of Hoffman is another inflammatory condition of the scalp that can lead to scarring alopecia. African Americans are primarily affected. This conditio n begins with deep inflammatory nodules, primarily over the occiput, that progress to coalescing regions of boggy scalp (Fig. 71-29). Sinus tracts may form and dislodge purulent material. As in folliculitis decalvans, S. aureus is the most common bacterial isolate. Biopsy of early lesions shows follicular plugging and suppurative follicular or perifollicular abscesses with a mixed inflammatory infiltrate of neutrophils, lymphocytes, plasma cells, or eosinophils.92,93 and 94 Later, foreign-body giant cells,

granulation tissue, and, finally, scarring with sinus tracts occur. Control is difficult to attain, but systemic steroids, systemic antibiotics, dapsone, or P.650

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retinoids109 are useful therapies. Surgical incision and drainage, excision with grafting, and/or x-ray epilation are occasionally used for refractory cases.

FIGURE 71-29 Dissecting folliculitis. ACNE KELOIDALIS Acne keloidalis (nuchae) is a destructive scarring folliculitis that occurs almost exclusively on the occipital scalp of African Americans, primarily men (Fig. 71-30). The clinically distinctive lesions begin as follicular pustules and papules and progress to persistent firm papules or coalesce into hairless keloid-like plaques.110 On histopathologic examination of an early lesion, there is follicular dilatation and a mixed peri-infundibular infiltrate that goes into follicular rupture and foreign body granulo mas, loss of sebaceous glands, and la mellar fibroplasia. Treatment with systemic

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antibiotics, topical and/or intralesional steroids, and cryosurgery is usually helpful.

FIGURE 71-30 Acne keloidalis. ACNE NECROTICA The primary lesion in acne necrotica is a pruritic or painful erythe matous follicular-based papule that develops central necrosis and crusting and heals with a varioliform scar.111 The lesions are concentrated on the nose, forehead and anterior scalp but may spread, primarily to the trunk. The course is chronic. Pathology is characterized by follicular dilatation, an early mixed lymphocytic/neutrophilic infiltrate in the peri-infundibulum, and later a lymphocytic/plasmacytic exocytosis perivascular individual and cell perifollicular necrosis of

infiltrate.

Lymphocytic

and

keratinocytes within the outer root sheath and surrounding epidermis go on to confluent necrosis of the central follicle. Treatment with tetracycline is generally helpful and cis-retinoic acid may be of value.111

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EROSIVE PUSTULAR DERMATOSIS Erosive pustular dermatosis of the scalp presents with pustules, erosions, and crusts on the scalp of primarily older Caucasean females ( Fig. 7131).112 On biopsy, there is a lymphoplasmacytic infiltrate foreign bod y giant cells and pilosebaceous atrophy. These lesions have a slow but progressive course. Multiple organisms, both bacterial and fungal, have been cultured but these probably represent secondary colonization; patients do not generally respond to antibacterial or antifungal drugs. Potent topical steroids, zinc sulfate, or isotretinoin may be helpful.

FIGURE 71-31 Pustular dermatosis of the scalp. Secondary Cicatricial Alopecia Cicatricial alopecia may present as a hereditary or development problem, alone or as part of a syndrome. Examples of the latter are ConradiHnermann chondrodysplasia punctata; incontinentia pigmenti;

ankyloblepharon, ectodermal defect, cleft lip or palate (AEC) syndro me;

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Hallermann-Streiff

syndrome;

and

generalized

atrophic

benign

epidermolysis bullosa.13 The most common congenital c icatricial alopecia is aplasia cutis congenita, which is the congenital focal absence of epidermis with or without absence of other layers of the skin.13 Hair follicles in the involved areas are variably affected. The condition may present at birth as an ulceration, crust, scar, or parchment-like membrane (Fig. 71-32). Eighty-five percent of aplasia cutis congenita presents on the scalp, and 70 percent of affected patients have only a single lesion. The lesions are usually small and round but can be large and extend to the dura or meninges. Aplasia cutis congenita may occur alone or in conjunction with various other

abnormalities. Unless the lesions of aplasia cutis congenita are very large, no specific treatment is needed.

FIGURE 71-32 Aplasia cutis congenita. (Photograph courtesy of Neil Prose, MD.)

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EXCESS HAIR Hirsutism refers to hair growth in women in areas of the body where hair growth is under androgen control and in which normally only postpubescent males have terminal hair growth. These areas include the moustache, beard, chest, escutcheon, and inner thigh. P.651

Hypertrichosis specifically refers to hair density or length beyond the accepted limits of normal for a particular age, race, or sex. The excess hair may be generalized or localized and may consist of lanugo, vellus, or terminal hair. Hirsutism Table 71-5 lists the causes of hirsutism. Most cases of hirsutism secondary to hyperandrogenism are associated with irregular menses or amenorrhea. There may be evidence of other cutaneous androgen-sensitive disorders such as acne and female pattern hair loss, or cutaneous clues to a related systemic problem such as acanthosis nigricans seen with insulin-resistant diabetes. Virilization is uncommon and should lead one to consider an underlying androgen-producing tumor.

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TABLE 71-5 Hirsutism For endogenous causes of hirsutism, a simple screening test of serum free or total testosterone will often determine whether further testing is necessary. Elevation of testosterone levels well above the upper limits of normal indicates the necessity of screening for an ovarian or adrenal tumor. Far more co mmon is the mild elevation of androgens in an otherwise healthy woman, which is most commonly secondary to either polycystic

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ovarian syndro me (PCOS) or late-onset congenital adrenal hyperplasia (CAH). PCOS is multifactorial, with a pituitary and gonadal component, as well as hyperinsulinemia that may lead to increased production of androgen, decreased production of estrogen, and anovulation. 113,114 The most common cause of late-onset CAH is 21-hydroxylase deficiency with overproduction of 17-hydroxyprogesterone.64 3-Hydroxylase and 11hydroxylase deficiency of may also present with late-onset hirsutism;

overproduction

17-hydroxypregnenolone

and

11-deoxycortisol,

respectively, occur with these enzyme deficiencies. The diagnosis of late onset CAH, while suggested by an elevated level of

dehydroepiandrosterone and testosterone, can only be established by a cosyntropin-stimulation test showing the expected rise in the specific steroid hormone that builds up immediately behind the enzyme blockade or deficiency.64 Wo men with hyperprolactine mia may have an increase in functional androgens through adrenal overproduction and through a decrease in sex hormone-binding globulin (SHBG) caused by a diminutio n of ovarian estrogen production; a prolactin level is diagnostic. 115 The effective treatment of hirsutism depends on the cause, but the mainstays of treatment are oral contraceptive pills, both for their direct effect on lowering androgen production and indirect effect on lowering androgen bioavailability by increasing SHBG, and for their contraceptive effect when used with antiandrogens or 5 -reductase inhibitors.

Antiandrogens, such as spironolactone, flutamide, or cyproterone acetate (see discussion of antiandrogens in Pattern Hair Loss, above), are

particularly useful in hirsutism as is finasteride.116 There are marginal differences in efficacy between these agents but all generally take 6 to 12

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months for sufficient miniaturization of terminal hairs to occur to be clinically significant. Treatment of congenital adrenal hyperplasia may also be accomplished through may be the use of low-dose with dexa methasone. either medical

Hyperprolactine mia

treated

directly

(bromocriptine) or surgical treatment of the hyperprolactino ma, and/or an antiandrogen may be utilized. Hypertrichosis GENERALIZED HYPERTRICHOSIS: INHERITED Congenital hypertrichosis lanuginosa presents as a confluent, generalized overgrowth of silvery blonde to gray lanugo hair at birth or in early infancy. It is rare (1 in 1 billion) and thought to occur as an autosoma l dominant trait with variable expressivity. In most cases, other than possibly anomalous dental eruptions, children are otherwise healthy. The hair may persist, increase, or decrease with age.117 There are several other congenital disorders associated with generalized hypertrichosis, but none that are so evenly distributed as congenital hypertrichosis lanuginosa. These are noted in Table 71-6.118 Patients with the autosomal dominant Ambras syndrome or hypertrichosis universalis congenita present with much longer, thicker hair, with accentuation over the entire face, ears, and shoulders.119 Associated facial dysmorphis m and dental anomalies are common. Members of the five-generation fa mil y described with congenital generalized hypertrichosis also have excess terminal hair on the face and upper body, more severe in men than women in keeping with the X-linked dominant inheritance.120 Patients with the autosomal dominant (rarely autosomal recessive) gingival fibromatosis frequently have hypertrichosis, mostly on the face, eyebrows, limbs, and

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upper back, along with seizures and oligophrenia.118 Hypertrichosis ma y be delayed until puberty although gingiva l fibromatosus in this situatio n usually appears with the emergence of the primary, versus secondary, teeth.

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TABLE 71-6 Causes of Generalized Hypertrichosis P.652

ACQUIRED GENERALIZED HYPERTRICHOSIS There are few more ominous signs in dermatology than the onset of generalized hypertrichosis without an obvious drug-related explanation. Acquired hypertrichosis lanuginosa almost always signals an underlying malignancy or is a harbinger of one to develop in the near future (Fig. 7133).121

FIGURE 71-33 Acquired hypertrichosis lanuginosa. (From Olsen,118 with permission.) There are several drugs that routinely cause generalized hypertrichosis (see Table 71-6).118 Oral minoxidil, a piperidinopyramidine derivative that is a

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potassium

channel

opener

and

antihypertensive

agent

when

used

systemically, causes hypertrichosis in 80 percent of patients, most prominently over the face, shoulders, and extremities. Diazoxide, a benzothiadiazine used primarily in malignant hypertension or idiopathic hypoglycemia of infancy, leads to lanugo-like hypertrichosis of the face, trunk, and extremities in 1 to 20 percent of adults but in almost 100 percent of children. Dilantin use leads to terminal hair hypertrichosis in 5 to 12 percent of patients, again first over the extremities, trunk, and face. Cyclosporine, a cyclic undecapeptide of fungal origin and an

immunosuppressive agent, causes terminal hair hypertrichosis in 40 to 95 percent of patients, with a more diffuse distribution of excess hair, but primarily over the upper body (Fig. 71-34).122,123 P.653

Both diazoxide and cyclosporine hyperplasia.

are also associated

with gingiva l

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FIGURE 71-34 Cyclosporine-related hypertrichosis. Certain medical illnesses are associated with widespread, although not confluent, hypertrichosis. They are listed in Table 71-6. Localized Hypertrichosis Table 71-7 lists the conditions associated with localized hypertrichosis. These are generally either inherited, develop mental, or secondary to irritation or trauma.

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TABLE 71-7 Causes of Localized Hypertrichosis Treatment of Hypertrichosis Removal of the inciting cause should be the prime approach to treatment and usually leads to regression of the hypertrichosis. However, in situations where that is not possible, one must either help the patient deal psychologically with the physical anomaly and/or use means of either temporarily or permanently removing the hair. Depilatories, plucking, waxing, and shaving are all means of te mporarily removing hair but, wit h the exception of shaving, may be associated with irritation and/or pseudofolliculitis barbae. Potentially, electrolysis can permanently remove unwanted hair, but this technique varies widely in effectiveness depending on the training of the electrologist, the type of machine, the pulse frequency, the intensity and duration applied, and the probe used. 1 Two main types of electrolysis are in general use, thermolysis (AC current) with destruction of the hair by local heat production and the blend, a

combination of thermolysis and galvanic (DC current) which produces destruction of the hair by local production of caustic lye and H 2 gas. Potential, but ultimately controllable, side effects of electrolysis are pain, scarring, infection, and folliculitis. Recently, several different kinds of laser have been approved for removal of hair.1 The lasers selectively target the hair follicle, either by targeting a chro mophore that is a natural co mponent of the follicle, such as melanin, or one that is introduced into the follicle. The ruby (694 nm), alexandrite (755 nm), and semiconductor diode (800 nm) lasers, as well as a flash-lamp device with filters able to deliver light of >590 nm, each target melanin in

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the hair shaft and cause selective thermal injury.1 The limiting factor in selecting melanin as the target is the concomitant absorption by epiderma l melanin with both potential epidermal injury and a diminution in energy dispersal down the hair follicle. The Q-switched neodymium:yttriumaluminum-garnet (Nd:Yag) laser with a wavelength of 1064 nm does not target melanin; instead, it targets a topically applied carbon-based material that leads to both thermal and mechanical damage to the follicle. All of the aforementioned lasers induce temporary hair removal. None of these techniques has been proven to lead to complete and permanent hair removal, although those that target melanin clearly can lead to a long-term reduction in terminal hair density, much apparently due to miniaturizat ion, versus destruction, of the follicles. 124 A more experimental treatment is photodynamic therapy based on a topical photosensitizer (aminolevulinic acid), and subsequent exposure to red light, which causes selective follicular damage by the synthesis of the potent photosensitizer,

protoporphyrin.1 CONCLUSION The range of abnormalities in hair disorders mirrors the co mplexities of hair production. The astute clinician is able to diagnose hair disorders by a combination of clinical clues, microscopic evaluation of hairs, a biopsy of the affected area, and confirmatory laboratory tests. Treatment efficac y mirrors diagnostic accuracy although we are currenly woefully short of treatment to help those patients with herediary disorders of hair follicle or shaft production. As we come to better understand the genetic and molecular controls on hair growth, earlier diagnosis and implementation of

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effective directed treatments for the primary disorders of hair loss or overgrowth will become possible. REFERENCES 1. Olsen EA: Methods of hair removal. J Am Acad Dermatol 40:143, 1999 2. Courtois M et al: Ageing and hair cycles. Br J Dermatol 132:86, 1995 3. Randall VA, Ebling FJG: Seasonal changes in human hair growth. Br J Dermatol 124:146, 1991 4. Olsen EA: Clinical tools for assessing hair loss, in Disorders of Hair Growth: Diagnosis and Treatment, edited by EA Olsen. New York, McGraw-Hill, 2003 5. Olsen EA: Current and novel methods for assessing efficacy of hair growth pro moters in pattern hair loss. Accepted for publication. J Am Acad Dermatol 48:253, 2003 6. Solomon AR: The transversely sectioned scalp biopsy specimen: The technique and the algorithm for its use in the diagnosis of alopecia. Adv Dermatol 9:127, 1994 P.654

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44. Elhassani SB: The many faces of methylmercury poisoning. J Toxicol 19:875, 1982 45. Stein KM et al: Toxic alopecia from ingestion of boric acid. Arch Dermatol 108:95, 1973 46. Bank WJ et al: Thallium poisoning. Arch Neurol 26:456, 1972 47. Price VH, Gummer CL: Loose anagen syndrome. J Am Acad Dermatol 20:249, 1989 48. Baden HP et al: Loose anagen hair as a cause of hereditary hair loss in children. Arch Dermatol 128:1349, 1992 49. Safavi K: Prevalence of alopecia areata in the First National Health and Nutrition Examination Survey. Arch Dermatol 128:702, 1992 50. Price VH: Alopecia areata: Clinical aspects. J Invest Dermatol 96:68S, 1991 51. Olsen EA et al: Alopecia areata investigational assessment guidelines. J Am Acad Dermatol 40:242, 1999 52. Muller SA, Winkelman RK: Alopecia areata. An evaluation of 736 patients. Arch Dermatol 88:290, 1963 53. Paus R et al: Is alopecia areata an autoimmune response against melanogenesis-related proteins exposed by abnormal MHC Class I

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melanocytes and melanosomes in acute alopecia areata. J Invest Dermatol 94:803, 1990 57. Colombe BW et al: HLA class II alleles in long standing alopecia totalis/universalis and long standing patchy alopecia areata differentiate these two clinical groups. J Invest Dermatol 104:4S, 1995 58. Ikeda T: A new classification of alopecia areata. Dermatologica 131:421, 1965 59. Olsen EA et al: Systemic steroids with or without 2% topical minoxidil in the treatment of alopecia areata. Arch Dermatol 128:1457, 1992 60. Claudy AL, Gagnaire D: PUVA treatment of alopecia areata. Arch Dermatol 119:975, 1983 61. Shapiro J: Topical immunotherapy in the treat ment of chronic severe alopecia areata. Dermatol Clin 11:611, 1993 62. Fiedler VC et al: Treat ment-resistant alopecia areata: Response to combination therapy with minoxidil plus anthralin. Arch Dermatol 126:756, 1990 63. Trakimas C et al: Clinical and histologic findings in temporal triangular alopecia. J Am Acad Dermatol 31:205, 1994 64. Olsen EA: Pattern ha ir loss, in Disorders of Hair Growth: Diagnosis and Treatment, edited by EA Olsen. New York, McGraw-Hill, 2003 65. Olsen EA: Fe male pattern hair loss. J Am Acad Dermatol 45:S70, 2001 66. Kaufman KD: Androgen metabolis m as it affects hair growth in androgenetic alopecia. Dermatol Clin 14:697, 1996

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67. Carey AH et al: Polycystic ovaries and premature male pattern baldness are associated with one allele of the steroid metabolis m gene CYP17. Hum Mol Genet 3:1873, 1994 68. Zhou Z-X et al: Specificity of ligand -dependent androgen receptor stabilization: Receptor domain interactions influence ligand dissociation and receptor stability. Mol Endocrinol 9:208, 1995 69. Bayne EK et al: Immunohistochemical localization of types 1 a nd 2 5-reductase in human scalp. Br J Dermatol 141:481, 1999 70. Sawaya ME, Price VH: Different levels of 5 -reductase type I and II, aromatase and androgen receptor in hair follicles of wo men and men wit h androgenetic alopecia. J Invest Dermatol 109:296, 1997 71. Kaufman KD et al: Finasteride in the treat ment of men with androgenetic alopecia. J Am Acad Dermatol 39:578, 1998 72. Price VH, Menefee E: Quantitative estimation of hair growth: Comparative changes in weight and hair count with 5% and 2% minoxidil, placebo and no treatment, in Hair Research for the Next Millenium, edited by D van Neste, VA Randall. Amsterdam, Elsevier Science BV, 1996, p 67 73. Unger WS: Surgical approach to hair loss, in Disorders of Hair Growth: Diagnosis and Treatment, edited by EA Olsen. New York, McGraw-Hill, 2003 P.655

74. Rushton DH et al: Quantitative assessment of spironolactone treatment in wo men with diffuse androgen-dependent alopecia. J Soc Cosmet Chem 42:317, 1991

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75. Cusan L et al: Treatment of hirsutism with the pure antiandrogen flutamide. J Am Acad Dermatol 23:462, 1990 76. Dawber RPR et al: Oral antiandrogen treatment of common baldness in women. Br J Dermatol 107(suppl 22):20, 1982 77. The Finasteride Male Pattern Hair Loss Study Group: Long-term (5year) multinational experience with finasteride 1 mg in the treatment of men with androgenetic alopecia. Eur J Dermatol 12:38, 2002 78. Rothbaum BO, Ninan PT: The assessment of trichotillomania. Behav Res Ther 32:651, 1994 79. Stein DJ et al: Trichotillo mania and obsessive-co mpulsive disorder. J Clin Psychiatry 56:28, 1995 80. Muller SA: Trichotillomania: A histopathologic study in sixty-six patients. J Am Acad Dermatol 23:56, 1990 81. Mehregan AH: Trichotillomania: A clinicopathologic study. Arch Dermatol 102:129, 1970 82. Elewski BE: Tinea capitis: A current perspective. J Am Acad Dermatol 42:21, 2000 83. Gan VN et al: Epide miology and treatment of tinea capitis :

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