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Dysfunction of Hair Growth
Dysfunction of Hair Growth CHARLES P. DEFEO, JR., M.D.*
Alopecia (hair loss) cannot be diagnosed unless one knows how scalp hair normally grows. Each hair passes through three stages of development: the anagen phase (growth phase) which may last for three to five years; the catagen or short phase (ten days to two weeks) in which hair growth activity stops; and the third stage or telogen. This stage produces resting hair in the follicle and new hair buds develop to replace hair which will be lost. This final stage may last for varying lengths of time and usually 15 per cent of the scalp's hair is in this resting stage at anyone time. Adjacent hairs are usually not in the same stage of maturation. Normally, people lose 40 to 90 hairs a day, but these are replaced by new hairs that are developing and there is no noticeable deficit. Hair problems are diagnosed best by breaking down hair loss into four groups: I. Il. Ill. IV.
Localized hair loss without scarring Localized hair loss with scarring Diffuse hair loss without scarring Diffuse hair loss with scarring
In some areas, one type of hair loss may merge with another, but this breakdown will at least give the observer a method of approach to the diagnosis. LOCALIZED NONSCARRING ALOPECIA
Alopecia Areata Alopecia areata (bald spot) is most commonly seen as a single circumscribed loss of hair from one area of the scalp without symptoms and the From the Department of Dermatology, New York University Schools of Medicine, New York, N.Y. * Assistant Clinical Professor of Dermatology, New York University Schools of Medicine; Attending Dermatologist, Misercordia Hospital; Associate Dermatologist, Lenox Hill Hospital and St. Clare's Hospital
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Figure 1. Forms of alopecia. A, A single lesion of alopecia areata. B, Alopecia totalis in a 5 year old boy that developed in six months. C, Alopecia areata with beginning hair regrowth. D, Alopecia areata resembling male pattern hair loss with the hair completely returning in 9 months.
hair regrows in a matter of months (Fig. 1, A). This simple variety may be complicated by more than one area of baldness, with some areas growing back while adjoining areas are still losing hair. The extreme variety progresses slowly or rapidly to involve the entire scalp hair and even all hair of the body. Some abnormal nail growth has been associated with this extreme condition in as high as 30 per cent of the cases. With alopecia areata in children, a poor prognosis can be expected because the condition in them is more likely to progress and they are more likely to have repeated attacks with a much higher incidence of persistent universal alopecia. 15 Familial incidence, from 10 per cent to 25 per cent of the cases studied, has been noted by most investigators. Alopecia was seen in a pair of identical twins beginning at the same time in the same location. 28 Alopecia areata in hairy areas other than the scalp offers a poorer prognosis. It is associated with an increased possibility of progression to total baldness (alopecia totalis) (Fig. 1, B).
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No consistent cause for alopecia areata has been found. Psychiatric disease has been found in 20 per cent of the cases reviewed at the Mayo Clinic, with acute emotional stress being the trigger mechanism in 12 per cent of the patients. No endocrinopathy of the pituitary, adrenal, thyroid and gonads has been shown to be consistently present. Muller and Winkelmann felt that the most likely cause was a "genetic influence in which there is a highly variable mode of inheritance, penetrance and expressivity of the genes" after they reviewed 736 cases at Mayo Clinic. 15 When advising an adult patient with a single patch of alopecia areata, one can afford to be optimistic about the hair returning. Caution should be used in predicting what will happen when there have been recurrent bouts, when there is more than one area involved, or when the alopecia has begun early in life. When hair starts to regrow, it usually begins in the center of the bald spot, showing up as thin, light colored hair (Fig. 1, C). With continued regrowth, the hair thickens and usually develops its normal color. Some areas may stay white or gray even though the hair establishes its normal length. Ophiasis is a word from antiquity which refers to the snake-like configuration of alopecia areata localized around the periphery of the hairy scalp. When this is seen, it is likely to be a harbinger of more severe and persistent hair loss. In a younger person it may be the beginning of total alopecia. In an adult it likewise has a poor prognosis for hair regrowth. When this type of nonscarring hair loss is seen, only a guarded prognosis can be made. Several areas of incomplete alopecia areata (Fig. 1, D) may develop on the anterior portion of the scalp in either males or females. This condition may be misdiagnosed as the usual male pattern hair loss or diffuse thinning in the female, but it differs from these in that it will respond to treatment and does not have the hopeless outlook of the other conditions. Therapy has consisted of many agents, most of them of questionable efficacy. Historically, x-ray, thorium X, ultraviolet light, caustic acids, hormones and sedatives have been used. In the solitary patch of alopecia areata it has been found that sublesional injection of triamcinolone acetonide (10 mg. per cc. in an aqueous suspension, injected 3 to 4 mm. below the skin in amounts of 0.1 to 0.2 cc. per injection site) may institute regrowth of hair in three or four weeks. 16 This same method of therapy has been tried with multiple injections in the more severe form of partial, total or universal alopecia with much less success. In this group of patients, internal corticosteroids have also been used with initially good results only to have the hair usually again fall out when medication was discontinued. Tinea Capitis (Ringworm of the Scalp)
A good rule of thumb should be to consider hair loss before puberty as being caused by ringworm until proved otherwise (Fig. 2). In most
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Figure 2. Ringworm of the scalp in several areas covered by a slight scale and hair loss.
cases of tinea capitis there are single or multiple circular lesions in which the hair is broken off. The hair loss may vary in configuration and the scalp is covered by slightly scaling patches with evidence of hairs piercing the scale, but being snapped off close to the scalp. This condition is caused by superficial fungi which attack the hair shaft itself and the surrounding skin. In the United States, the two most common species of fungi which cause this picture are Microsporum and Trichophyton; the more common varieties include Microsporum audouini and Microsporum canis. These two species generally cause fluorescence of the involved hairs under an ultraviolet light emitting wave lengths of 3650 Angstroms. This may be obtained by using a Wood's light filter. Not all hairs infected with a fungus will fluoresce under this light, but it is certainly a good screening device in checking school children or other members of the family who have been exposed to ringworm. Apparently, the fungus must not only be placed upon the scalp by direct contact or contact with a comb or brush from an infected person, but there must be some injury to the scalp allowing the fungus to gain a foothold. These fungus infections particularly involve children in urban areas where they are in close contact with each other and more commonly involve boys than girls. Uncommonly, these fungus infections of the scalp may call forth a marked reaction called a kerion in which a pustular response develops in a localized area, which often looks like a bacterial infection. This reaction cures itself spontaneously, but may, if the involvement is severe enough, cause scarring and permanent hair loss. In any event, most fungus infections will spontaneously disappear in time if left alone, and will disappear at puberty if the infection is encountered late in childhood. Several uncommonly encountered fungi may cause tinea capitis. They are Trichophyton violaceum, Trichophyton schoenleini and Trichophyton
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tonsurans. The important characteristic of this group is that these fungi do not routinely fluoresce under a Wood's light. Thus, a patient with this type of infection will not be detected if checked only by this method. It is therefore wise to check any patient who has a scaling patch of alopecia by pulling out several of the hairs, looking at them under a microscope directly and culturing them to spot the offending fungus. A positive microscopic diagnosis may be obtained using a 15 per cent solution of sodium hydroxide on several infected hairs. This mixture is warmed slightly, macerating the hairs, and direct examination with the microscope will reveal spores and hyphae. In a differential diagnosis of ringworm of the scalp, one should consider impetigo, alopecia areata and seborrheic dermatitis. Alopecia areata may be differentiated because of the smooth, shiny surface of the scalp, showing no evidence of scaling or inflammation. Seborrheic dermatitis (cradle cap) sometimes causes circular scaling patches on the scalp without hair loss. Impetigo patients usually show heaped-up yellowish crusts on their scalps without evidence of alopecia. Treatment of tinea capitis should consist of griseofulvin tablets given orally in a dosage between 250 and 500 mg. daily, depending upon the weight of the child. Half of this daily dose, 125 to 250 mg., is necessary with ultrafine griseofulvin. These can be taken following the largest meal since fats enhance absorption. Therapy should continue four to six weeks. Shaving the scalp when feasible prevents dissemination to other children and applying adhesive tape across the involved area daily removes infected hairs. In Trichophyton infection, the daily dosage of griseofulvin should be doubled and continued until there is no microscopic evidence of continued infection. (See also Dr. Reiss's article on fungus infections.)
Trichotillomania This is a behavior pattern. Such an individual deliberately pulls the hair out from a given region of the body, either the scalp, eyebrows, eyelashes or body hairs. The exact mental mechanisms causing this behavior are difficult to ascertain, but the individual will pluck the hair out from a given irregularly outlined area. The diagnostic clue will be a scattering of hairs too short to be pulled out, which is in contradistinction to alopecia areata in some patients who may have evidence of hair present but it will be in the form of hairs easily tweezed out. There is usually no evidence of inflammation and the scalp appears completely normal. This type of selfdestruction is not to be taken as lightly as finger sucking and nail biting. It has a more deep-seated prognostic outlook; often psychiatric help may be necessary. These people are not the ones who unconsciously rub or pluck a given area while concentrating or studying. The latter will come in with the hairs sheared off or pulled out in a small region. This is merely a habit, perhaps hard to change, but it is not a difficult psychiatric problem.
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Figure 3. Traumatic marginal alopecia.
Traction Alopecia TRAUMATIC MARGINAL ALOPECIA. This is a form of temporary or permanent hair loss commonly seen in Negroes (Fig. 3). It is produced by excessively tight braiding of the hair24 which, because of continuous pressure on the roots, causes it to fall out, generally around the edges of the pleated area. Often this is reversible if the braiding is discontinued early. In some cases, however, secondary bacterial infection will cause permanent hair loss. PONYTAIL ALOPECIA. Now that ponytail hair styling has become passe, this type of alopecia has become rare. When ponytails were frequently used by women, the constant pressure of the tightly pulled hair often resulted in the appearance of alopecia around the peripheral areas, usually the front and sides of the scalp. This was caused by the same mechanism that produces traumatic marginal alopecia. ROLLER BRUSH ALOPECIA. This is a type of alopecia generally seen along the vertex of the scalp when females use roller brushes to curl their hair. 13 By doing this, they put undue pressure on the hair, and it may be either pulled out in multiple areas along the vertex or snapped off just above the scalp. In all cases, this condition is completely reversible if this type of hair curling is discontinued. However, these brush rollers may stick into the scalp to cause a low grade folliculitis, which may complicate the diagnosis. Pressure Alopecia This is a type of alopecia originally described by Abel and Lewis. l They reported eight cases that appeared following protracted surgical procedures in which the head was held in one position. This type of alopecia usually appears several days after the operation in the form of a soft, moist
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area on the posterior aspect of the scalp generally involving no more than a silver dollar sized area j it looks like localized folliculitis. Many times this initial stage of the alopecia is missed since the patient, having undergone extensive surgery, is under considerable sedation. It first resembles alopecia areata, but later usually takes on an irregular outline showing snapped off hairs at the periphery. There may be some hyperpigmentation at the time the patient first consults the physician since the original dermatitis has subsided. An excellent prognosis may be offered since hair completely returns without therapy.
Postfuruncular Alopecia Postfuruncular alopecia3 follows the development of single or multiple furuncles in the scalp. The alopecia is, in this case, confined to the region of the elevation caused by the furuncle. The hair loss begins shortly after the infectious process starts and the prognosis is excellent. It is caused by pressure from below upon the pilosebaceous structure causing hair to fall out. No therapy is required.
Tick-Bite Alopecia In several western states, it has been reported that tick bites produce inflammatory dermatitis,21 which may subsequently affect the deeper scalp structures causing a transient localized area of alopecia. This resembles alopecia areata or the "moth-eaten" alopecia of secondary syphilis when there has been more than one tick bite.
Figure 4.
Alopecia mucinosa with follicular spines in the areas of alopecia.
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Alopecia Mucinosa This rare disease generally arises with an area of alopecia some place on the scalp-but not always (Fig. 4).19 If it arises in the scalp, there are follicular papules in the center of an area of baldness. The hair loss is primarily due to mucinous changes in epithelial cells of the pilosebaceous follicle with secondary loss of hair. It is now believed that this mucinous degeneration in the hair follicles may accompany several basic conditions, one of which may be self-limited and may spontaneously regress. The condition may also be seen in other inflammatory lesions of the scalp and sometimes in mycosis fungoides. In the latter condition, the lesions on the scalp will respond to x-radiation, but will recur. More investigation is necessary on the pathophysiology of this condition.
LOCALIZED SCARRING ALOPECIA
Discoid Lupus Erythematosus Clinically, scalp lesions generally appear as areas of alopecia showing some redness of the bald area in their initial stages and a minimal amount of scaling and follicular plugging. The alopecia is permanent and healing takes place with scarring and hypo- or hyperpigmentation. Old· healed areas of lupus erythematosus showing atrophy may be adjacent to areas of activity. Usually, one is not confronted with a diagnosis of only lesions on the scalp i often other lesions appear on the light-exposed portions of the trunk, hands and ears. In Negroes, older areas may exhibit white, atrophic, depigmented areas with a hyperpigmented festooned border in which the active process is still present. Biopsy, generally of the active area on the scalp, usually leads to proper diagnosis. Frequently, the problem of differentiating discoid lupus erythematosus from acute disseminated lupus erythematosus is difficult. Other dermatoses resembling this are seborrheic dermatitis, polymorphous light sensitivity and occasionally psoriasis. Therapy for discoid lupus erythematosus of the scalp is the same as elsewhere on the body. The antimalarial drugs (chloroquine tablets) may be prescribed. They should be given in a dosage of 250 mg. once or twice a day for three to four weeks. Continuous therapy with high doses for long periods should be avoided because damage to the cornea and retina have been noted. With some of the newer steroids, topical and sublesional therapy has proved effective when they are injected 3 mm. below the surface of the skin. Triamcinolone acetonide, 0.1 cc. in each centimeter of skin showing lupus erythematosus, has proved helpful. The application of the same agents topically under occlusive therapy (using polyethylene wrap or Blenderm tape) often leads to a resolution where in the past other topical modalities were not effective.
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Scleroderma Scleroderma, when it involves the scalp and causes alopecia, generally takes one of two forms. The first is a linear midline lesion extending onto the forehead. It causes complete absence of hair in the involved area, resulting in the classical coup de sabre appearance. Most often this begins in the first decade of life. The area shows a sclerotic patch which is bound down to underlying structures. There is no hair growing in the area, which is depressed below the skin level. This may be associated with facial hemiatrophy. The second variety is morphea (localized scleroderma) which appears as an edematous patch surrounded by a violaceous halo. As time passes, the area loses its acute activity and becomes bound down with the classical ivory color seen in morphea. Most patches involute spontaneously, leaving permanent baldness. No therapy for either condition is known to be effective.
Pseudopelade This is a rare, scarring, progressive alopecia which appears as an area of atrophy (Fig. 5). Upon inspection, no activity is seen around edges of the areas of alopecia to account for the hair loss. The condition usually begins as a small area on the top of the head near the vertex which slowly progresses giving an irregular outline to the alopecia. It looks like "footprints in the snow." The involved area shows thinning skin and a loss of subcutaneous scalp tissue so that the area is depressed below the skin level. This and other scarring alopecias may, when the disease process has burned out, take on this "footprint" appearance. Lupus erythematosus, folliculitis decalvans and lichen planopilaris must be considered in the diagnosis. Therapy, when scarring becomes too noticeable, consists of grafting other scalp hair into the area of alopecia.
Figure 5. Pseudopelade showing the irregular outline of alopecia, scarred surface, and no inflammation around the hair follicles at the periphery.
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Alopecia due to excess ionizing radiation.
Lichen Planopilaris This is an unusual and rare variety of lichen planus which produces small elevated papules about the hair follicle. It may involve the scalp, resulting in irregularly outlined areas of alopecia with central atrophic whitish scars. There may be similar small grouped papules on the body giving a clue to the diagnosis. This condition is different from folliculitis decalvans and pseudopelade of the scalp, but they all eventuate into similar scarring alopecia. No therapy is effective.
Injury, Burns, and Ionizing Radiation Injuries to the scalp deep enough to avulse the entire hair-bearing area if not replaced adequately, or if there is not enough tissue to cover the defect, will heal with scarring leading to irregularly outlined areas of alopecia. Burns which destroy the full thickness of skin lead to permanent scarring and alopecia. X-ray burns causing permanent alopecia and scarring may develop if more than 1200 roentgens of superficial x-ray are given to anyone region of the scalp (Fig. 6). Broad telangiectasias course across the area of baldness. The hair loss is usually not complete because less radiation reaches the periphery of the scalp.
Bacterial Infection, Furuncles, Carbuncles and Acne Keloidalis Furuncles and carbuncles of the scalp, especially those localized in the suboccipital region, will heal with irregular scars without hair. In some individuals, especially Negroes, there may be an excessive amount of scar tissue resulting in acne keloidalis. This causes scarring in the nuchal region with erythematous follicular keloids elevated above skin level, leading to permanent alopecia in the area involved. Therapy includes, in these cases, the production of adequate drainage of the lesion and systemic administration of appropriate antibiotics. Acne keloidalis may require dry ice application to flatten excessive scar tissue. Intralesional injection
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of corticosteroids may also be effective when given in a fashion similar to the treatment noted under alopecia areata.
Minor Ectodermal Defects Occasionally one may see a newborn infant with a small, shiny erythematous patch across the parietal area of the scalp} It may range in size up to a diameter of 3 cm., and there is no hair present. These defects are often believed to be caused by the use of instruments at the time of delivery, but in reality they are small ectodermal defects. As time passes, the area becomes depressed below skin level and .shows no tendency for hair growth. If one sees a minor ectodermal defect, it is wise to be certain there are no other ectodermal malformations in the child. No therapy is necessary because the adjacent hair usually covers the defect.
Primary and Metastatic Neoplasia of the Scalp Basal cell carcinomas have at times caused areas of alopecia usually beginning on the light-exposed areas of the forehead or on the bald pate. The carcinoma gradually enlarges through the years, forming an active rolled border with complete destruction of the hair roots and leading to baldness in the involved regions. These are nonmetastasizing tumors and can usually be eradicated either by surgery or x-ray. Metastatic carcinoma may also involve the scalp and cause alopecia;5, 20 by far the most common site of the primary tumor is the breast. The affected scalp takes on a fairly characteristic appearance in which there is a slowly progressing area of alopecia showing a cobblestone appearance. Occasionally, this may be mistaken for alopecia areata, or the bound-down appearance of morphea (localized scleroderma). A history of previous surgery on a malignant breast tumor can be
Figure 7. Folliculitis decalvans with its characteristic infected hair follicles.
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helpful in making the diagnosis, but there have been reports of the metastasis appearing in the scalp before the primary nodule was discovered in the breast.
Folliculitis Decalvans This is also a rare form of scarring alopecia caused by the production of small follicular pustules leading to destruction of the pilosebaceous apparatus (Fig. 7). Usually, activity begins in a small area with central atrophy and then spreads in a centrifugal fashion so that the folliculitis is seen at the periphery of the lesion. The outline is irregular and not every hair in the patch of alopecia may be affected. Therapy in this condition should include adequate systemic antibiotics and topical antibiotics under occlusive polyethylene wrap therapy. The end result without treatment or with unsuccessful treatment is that of irregularly outlined areas of alopecia with smooth, depressed scarring in the center of the lesion.
DIFFUSE NONSCARRING ALOPECIA
Male Pattern Hair Loss
This is the usual hair loss seen in young males. It generally begins in the early twenties when there is an elevation of the anterior hair line forming the classic "widow's peak." A genetic predisposition plus the development of a normal amount of androgenic hormones are essential for its progression. It has long been known that people who are castrated before the age of puberty, no matter what their genetic predisposition, do not develop this type of alopecia. It is interesting that androgenic stimulation tends to cause an increase in growth of the hairs on the rest of the body. It is paradoxical that only hair follicles on top of the scalp seem to have genetic predisposition for hair loss. Persons who suffer from male pattern hair loss may also suffer from dandruff. It has been proposed, but not proved, that the unsaturated acids, squalene and oleic acids, found in sebum may have some effect in causing increased hair loss in both sexes. 7 All the other causes suggested for this type of hair loss, i.e., decreased blood supply, 6 tight scalp, and a lack of subcutaneous fat on the scalp, have been disproved. Therapy is unsuccessful. Female Hair Loss or Thinning
In the past few years there have been increasing complaints of hair thinning in women. 4 , 25 Studies have failed to reveal any true cause for this particular type of thinning which has much the same pattern as that of the male who is progressing to complete baldness across the vertex of the scalp. Women usually have thinning only in comparable areas and generally it does not progress to severe alopecia. Nevertheless, this problem has been increasingly perplexing to the dermatologist; anyone confronted with it learns that the loss of woman's crowning glory throws fear into their hearts. Many theories as to the cause of this condition have been put forth, including the idea of it being due to hair dyes, permanent waves,
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teasing, wave sets, or hair sprays. Nylon brushes sometimes cause a snapping off of the hairs and produce thinning. Extensive endocrinological work-up of the pituitary, thyroid, adrenal and ovary has failed to reveal any cause. Some question has been raised as to whether there is a true increased incidence in this condition or merely an increased awareness, since the female is now more devoted to the adoration of her hair.26 It may be that sometime during the adult life of the female, her normal hair count undergoes a decrease, establishing a new norm. When this occurs, she is emotionally disturbed out of all proportion to the hair loss. Elderly women occasionally will have extensive thinning across the vertex, but generally by that time they are not worried about their physical appearance. Many times the normal hair fall of between 40 to 90 hairs a day will have been re-established, yet the individual is counting every hair as it comes out and worrying. In most cases, extensive reassurance of the patient is all that is required. Treatment is directed at ascertaining that the patient is not causing, through some manipulation, the snapping off of her hair at varying lengths above her scalp either by using hair straighteners or curling rollers, or by performing some other traumatic manipulation. These can produce hair thinning that is merely the snapping off of the distal tips. Also, an endocrinological work-up, although rarely rewarding, should be done.
Total and Universal Alopecia Alopecia totalis may be the end result of alopecia areata (Fig. 1, B). In 20 per cent of 736 cases at the Mayo Clinic,!" five or more years elapsed before development of complete hair loss. Ordinarily the loss progresses to a total loss at a slower rate in children than in adults, but its occurrence is more frequent. Multiple areas of alopecia, involving hairy areas other than the scalp, portends to a more severe and chronic form with an increased likelihood of progression to total alopecia. In the totalis group, 20 per cent of the children and 30 per cent of the adults in the Mayo series showed no significant regrowth. Only about 1 per cent of the children and 10 per cent of the adults showed permanent regrowth. It should be emphasized that approximately 40 per cent of the children and 30 per cent of the adults who developed total alopecia had subsequent periods of normal or near normal hair growth. In these groups of patients with alopecia totalis or universal alopecias there is an increased incidence of cataracts. These cataracts were seen in 7 of the 736 cases at the Mayo Clinic. l5 These patients were mainly in their fourth and fifth decades and had a posterior subcapsular type of cataract.
Alopecia of the Newborn A large number of newborn infants between one and four months of age have a bald area, most often across the posterior aspect of the scalp.
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This is believed to be caused by frictional rubbing of the scalp against the bed or cradle, but this is not usually true since most infants lose their first growth of hair completely during the first six months of life. 12 If this hair loss, which takes place as a normal physiological mechanism, occurs in sharp waves, there may be complaints from mothers that the child has developed alopecia. For the most part, this goes unnoticed because there is already some asynchronism of each hair follicle. This type of hair loss is generally unnoticeable by the time the child is six months old. Drug-Induced Alopecia
The following drugs have been shown to cause alopecia: nitrogen mustard, heparin and other anticoagulants, thallium and all the anticancer drugs, particularly the folic acid antagonists. Several pathological mechanisms account for drug-induced hair loss. In heparin alopecias there is a sudden increased number of resting hairs (telogen stage), so that a latent period of from one to four months is necessary before the alopecia develops. Since this type of hair loss may be phasic, and each follicle is in a different phase, it is understandable why many physicians never notice it. This same mechanism holds true with all anticoagulant drugs. With cancericidal drugs another mechanism prevailsY Here growing hair (anagen stage) is lost. Depending upon the dose of the cancericidal drug, the entire activity of hair growth may be suspended. If the insult is sufficient, as much as 80 or 90 per cent of the hairs are lost in a wave-like fashion, generally beginning with a short latent period of one to three weeks. If the insult is not sufficient, the hair will not be lost but will merely show a narrowing hair shaft. This likewise happens with nitrogen mustard, thallium, and folic acid antagonists. Postfebrile Hair Loss
Only occasionally in this era of antibiotics do individuals run a high enough temperature to cause hair fall. It may follow any febrile illness, usually pneumonia, in which the temperature is elevated above 103° F. for a long period.This temperature causes an increased number of the hairs to progress to the resting stage (telogen phase) and they usually begin to be shed two to three months after the illness, with most of the shedding being completed in six to eight weeks. There is no area of localization of the alopecia and usually all of the hair will regrow. Therapy should consist of reassuring the patient that all of the hair will return in time. Secondary Syphilis
It has long been known that syphilis, in its secondary stages, may cause an alopecia in which there is hair loss in individual areas which are
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generally spread across the scalp, but classically are located around the sides of the head. The clinical appearance is "nlOth-caten." The clue to this diagnosis is the presence of other manifestations of secondary syphilis such as a generalized maculopapular eruption. The quantitative serum test for syphilis (STS) is always elevated. Once the individual has been treated with adequate amounts of penicillin, there is complete restitution of this hair and no permanent alopecia. Postpartum Hair Loss
Most women know that many members of their sex complain of increased hair loss following childbirth, only to have it return to normal. Lynchfield followed a group of normal women through pregnancy and found that they developed an increased percentage of resting hairs which eventually fell out about two or three months following delivery. There was noticeable thinning in four of the 26 patients and he noted that roughly 40 to 50 per cent of the hairs must be lost before visible thinning is noted on the scalp. There is some good evidence that during the second and third trimesters of pregnancy fewer hair follicles enter into the resting stage. As a consequence, anywhere from two to three months postpartum there is an accelerated increase in resting hairs which are subsequently lost. Thus, as previously stated, even though there lllay be a complaint by the woman who is pregnant that she is losing her hair and it seems to be somewhat thinner, there is no sound evidence pointing to any significant regions of alopecia. Schiff and Kern found hair loss usually began by the tenth to thirteenth week postpartum and the majority of the women in their series had noted their hair fall by the twentieth week. 23 It was also noted that most of these persons had complete restitution of hair after several months. There is weighty evidence for a hormonal factor in the causation of postpartum hair loss, the most pressing one being that if women who had had multiple pregnancies and suffered from repeated postpartum hair loss became pregnant again before the tenth to thirteenth week, they did not subsequently develop postpartum alopecia. The prognosis is excellent for complete restitution of all of the hair that was lost postpartum. Congenital Alopecias
Congenital alopecia is the absence of hair or development of extremely poor hair on the scalp or on the rest of the body (Fig. 8). The infant may never have any hair, or the first hair may develop normally only to fall out. Subsequent growth of hair is shorter, lighter, and the hairs are fewer in number than normal. Most of these people have normal teeth and nails and enjoy good health. There are apparently two modes of transmission of this characteristic:
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Figure 8. Congenital alopecia in 2 generations.
in some families it is transmitted as a dominant gene and in others as a recessant gene. No therapy is of any avail. Monilethrix is a rare condition in which the hair shaft has alternate normal areas and thinned areas so that the hair appears beaded (Fig. 9). As a result, the hairs tend to snap off before they reach normal length. The appearance of the scalp is one of a crewcut or of a generalized diffuse hair loss. The condition is most likely to appear on the posterior aspects of the scalp, but may involve any area of the body. It is usually associated with follicular hyperkeratotic papules in the involved areas and on adjacent areas of the neck and upper extremities. This condition may arise at any time in life. Usually it begins prior to the first year, but may begin at any time-particularly at puberty. The diagnosis is simple, once one suspects it, in that a microscopic examination of the hair will alternately show a zone of thin shafts next to a zone of normal hair shafts. This has been reported as a dominant hereditary condition since several members of the same family may have it; others report it as a completely recessive trait. Monilethrix must be differentiated from pili annulati or "ringed hairs." This condition shows alternate light and dark bands in the hair with no alteration in the circumference. This phenomena is caused by air in the matrix of the hair in light areas. Monilethrix must also be differentiated from trichorrhexis nodosa. This
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Figure 9. Monilethrix. Congenital beading of the hair leads to its easy fracture so that it appears the hair has been cut short.
condition has a tendency to fracture the hair shaft so that there are tuftlike breaks in the shaft of the hair, causing it to snap off more easily. The usual causes are excessive manipulation of the hair, excessive brushing, or some sort of mechanical or beautifying destruction of the normalconfiguration of the hair shaft. There is no therapy for the first two conditions. In the trichorrhexis nodosa, extreme caution should be exercised in the way the hair is coiffured. DIFFUSE SCARRING ALOPECIA PERIFOLLICULITIS CAPITIS ABSCEDENS ET SUFFODIENS (Dissecting Folliculitis of the Scalp). Some individuals develop perifolliculitis of the scalp. Multiple draining sinuses generally involve a large portion of the scalp so that the entire area is one mass of necrotic draining lesions interconnecting to form a boggy mass across the area of the scalp. Many of the patients have comedones (blackheads) interspersed in each of the hair follicles, often associated with acne elsewhere on the body. Therapy should consist of the intensive use of external and internal antibiotics. When an extended area of the scalp is involved, temporary x-ray epilation of the scalp may at times be necessary to arrest this troublesome process when all other methods have failed. It is evident that those conditions discussed under localized scarring alopecia may in the severe case cause a diffuse scarring alopecia.
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REFERENCES 1. Abel, R. R. and Lewis, G. M.: Postoperative (pressure) alopecia. A.M.A. Arch. Dermat. 81: 34-41, 1960. 2. Andrews, G. and Domonkos, A.: Diseases of the Skin. Philadelphia, W. B. Saunders Co., 1963. 3. Butterworth, T. and Fowler, J. C.: Postfuruncular alopecia. A.M.A. Arch. Dermat. 80: 570-573, 1959. 4. Butterworth, T. and Strean, L. P.: Clinical Genodermatology. Baltimore, Md., Williams & Williams Co., 1962. 5. Cohen, I., Levy, E. and Schreiber, H.: Alopecia neoplastica due to breast carcinoma. Arch. Dermat. 84: 490-492, 1961. 6. Cormia, F. E. and Ernyey, A.: Circulatory changes in alopecia. Arch. Dermat. 84: 772-789, 1961. 7. Flesch, P.: Hair loss from sebum. A.M.A. Arch. Dermat. 66: 1-9, 1953. 8. Gentles, J. C. and Barnes, B. A.: A report on animal experiments with griseofulvin. A.M.A. Arch. Dermat. 81: 703-708, 1960. 9. Guy, W. B. and Edmundson, W. F.: Diffuse cyclic hair loss in women. A.M.A. Arch. Dermat. 81: 205-207, 1960. 10. Haynes, H. A., Jr. and Parry, T. L.: Alopecia areata associated with refractive errors. Arch. Dermat. & Syph. 59: 340-343, 1949. 11. Kligman, A. M.: The human hair cycle. J. Invest. Dermat. 33: 307,1957. 12. Kligman, A. M.: Pathological dynamics of human hair loss. Arch. Dermat. 83: 175, 1961. 13. Lipnik, M. L.: Traumatic alopecia from brush rollers. Arch. Dermat. 84: 493-495, 1961. 14. Montagna, W.: The Structure and Function of Skin. New York, Academic Press, Inc., 1958. 15. Muller, S. A. and Winkelmann, R. K.: Alopecia areata-an evaluation of 736 patients. Arch. Dermat. 88: 290-297, 1963. 16. Orentreich, N., Sturm, H. M., Weidman, A. I. and Pelzig, A.: Local injection of steroids and hair regrowth in alopecias. A.M.A. Arch. Dermat. 82: 894-902, 1960. 17. Pecoraro, V., Astore-Ignacio and Barman, J. M.: Cycle of the scalp hair of a newborn child. J. Invest. Dermat. 43: 145, 1964. 18. Pillsbury, D. M., Shelley, W. B. and Kligman, A. M.: Dermatology. Philadelphia, W. B. Saunders Co., 1956. 19. Pinkus, H.: Alopecia mucinosa. A.M.A. Arch. Dermat. 76: 419-426,1957. 20. Ronchese, F.: Alopecia due to metastases from adenocarcinoma of the breast. Arch. Dermat. & Syph. 59: 329-332, 1949. 21. Ross, M. S. and Friede, H.: Alopecia due to tick bite. Arch. Dermat. 87: 609-611, 1963. 22. Savill, A. and Warren, C.: The Hair and Scalp. Baltimore, Md., Williams & Williams Co., 1962. 23. Schiff, B. L. and Kern, A. B.: Postpartum alopecia. Arch. Dermat. 87: 609-611, 1963. 24. Slepyan, A. H.: Traction alopecia. A.M.A. Arch. Dermat. 78: 395-398, 1958. 25. Smith, M. A. and Wells, R. S.: Male-type alopecia, alopecia areata, and normal hair in women. Arch. Dermat. 89: 95-98, 1964. 26. Sulzberger, M. B., Witten, V. H. and Kopf, A. W.: Diffuse alopecia iI'l women. A.M.A. Arch. Dermat. 81: 556-560,1960. 27. Van Scott, E. J., Reinertson, R. P. and Steinmuller, R.: The growing hair roots of the human scalp and morphologic changes therein following Amethopterin therapy. J. Invest. Dermat. 29: 197, 1957. 28. Weidman, A. I., Zion, L. S. and Mamelok, A. E.: Alopecia areata occurring simultaneously in identical twins. A.M.A. Arch. Dermat. 76: 424-425, 1956. 29. Winkelmann, R. K. and Jaffe, M. 0.: Nerve network of the hair follicle in alopecia areata. A.M.A. Arch. Dermat. 82: 750-753, 1960.
140 East 54th Street New York, N.Y. 10022
Alopecia (hair loss) cannot be diagnosed unless one knows how scalp hair normally grows. Each hair passes through three stages of development: the anagen phase (growth phase) which may last for three to five years; the catagen or short phase (ten days to two weeks) in which hair growth activity stops; and the third stage or telogen. This stage produces resting hair in the follicle and new hair buds develop to replace hair which will be lost. This final stage may last for varying lengths of time and usually 15 per cent of the scalp's hair is in this resting stage at anyone time. Adjacent hairs are usually not in the same stage of maturation. Normally, people lose 40 to 90 hairs a day, but these are replaced by new hairs that are developing and there is no noticeable deficit. Hair problems are diagnosed best by breaking down hair loss into four groups: I. Il. Ill. IV.
Localized hair loss without scarring Localized hair loss with scarring Diffuse hair loss without scarring Diffuse hair loss with scarring
In some areas, one type of hair loss may merge with another, but this breakdown will at least give the observer a method of approach to the diagnosis. LOCALIZED NONSCARRING ALOPECIA
Alopecia Areata Alopecia areata (bald spot) is most commonly seen as a single circumscribed loss of hair from one area of the scalp without symptoms and the From the Department of Dermatology, New York University Schools of Medicine, New York, N.Y. * Assistant Clinical Professor of Dermatology, New York University Schools of Medicine; Attending Dermatologist, Misercordia Hospital; Associate Dermatologist, Lenox Hill Hospital and St. Clare's Hospital
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Figure 1. Forms of alopecia. A, A single lesion of alopecia areata. B, Alopecia totalis in a 5 year old boy that developed in six months. C, Alopecia areata with beginning hair regrowth. D, Alopecia areata resembling male pattern hair loss with the hair completely returning in 9 months.
hair regrows in a matter of months (Fig. 1, A). This simple variety may be complicated by more than one area of baldness, with some areas growing back while adjoining areas are still losing hair. The extreme variety progresses slowly or rapidly to involve the entire scalp hair and even all hair of the body. Some abnormal nail growth has been associated with this extreme condition in as high as 30 per cent of the cases. With alopecia areata in children, a poor prognosis can be expected because the condition in them is more likely to progress and they are more likely to have repeated attacks with a much higher incidence of persistent universal alopecia. 15 Familial incidence, from 10 per cent to 25 per cent of the cases studied, has been noted by most investigators. Alopecia was seen in a pair of identical twins beginning at the same time in the same location. 28 Alopecia areata in hairy areas other than the scalp offers a poorer prognosis. It is associated with an increased possibility of progression to total baldness (alopecia totalis) (Fig. 1, B).
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No consistent cause for alopecia areata has been found. Psychiatric disease has been found in 20 per cent of the cases reviewed at the Mayo Clinic, with acute emotional stress being the trigger mechanism in 12 per cent of the patients. No endocrinopathy of the pituitary, adrenal, thyroid and gonads has been shown to be consistently present. Muller and Winkelmann felt that the most likely cause was a "genetic influence in which there is a highly variable mode of inheritance, penetrance and expressivity of the genes" after they reviewed 736 cases at Mayo Clinic. 15 When advising an adult patient with a single patch of alopecia areata, one can afford to be optimistic about the hair returning. Caution should be used in predicting what will happen when there have been recurrent bouts, when there is more than one area involved, or when the alopecia has begun early in life. When hair starts to regrow, it usually begins in the center of the bald spot, showing up as thin, light colored hair (Fig. 1, C). With continued regrowth, the hair thickens and usually develops its normal color. Some areas may stay white or gray even though the hair establishes its normal length. Ophiasis is a word from antiquity which refers to the snake-like configuration of alopecia areata localized around the periphery of the hairy scalp. When this is seen, it is likely to be a harbinger of more severe and persistent hair loss. In a younger person it may be the beginning of total alopecia. In an adult it likewise has a poor prognosis for hair regrowth. When this type of nonscarring hair loss is seen, only a guarded prognosis can be made. Several areas of incomplete alopecia areata (Fig. 1, D) may develop on the anterior portion of the scalp in either males or females. This condition may be misdiagnosed as the usual male pattern hair loss or diffuse thinning in the female, but it differs from these in that it will respond to treatment and does not have the hopeless outlook of the other conditions. Therapy has consisted of many agents, most of them of questionable efficacy. Historically, x-ray, thorium X, ultraviolet light, caustic acids, hormones and sedatives have been used. In the solitary patch of alopecia areata it has been found that sublesional injection of triamcinolone acetonide (10 mg. per cc. in an aqueous suspension, injected 3 to 4 mm. below the skin in amounts of 0.1 to 0.2 cc. per injection site) may institute regrowth of hair in three or four weeks. 16 This same method of therapy has been tried with multiple injections in the more severe form of partial, total or universal alopecia with much less success. In this group of patients, internal corticosteroids have also been used with initially good results only to have the hair usually again fall out when medication was discontinued. Tinea Capitis (Ringworm of the Scalp)
A good rule of thumb should be to consider hair loss before puberty as being caused by ringworm until proved otherwise (Fig. 2). In most
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Figure 2. Ringworm of the scalp in several areas covered by a slight scale and hair loss.
cases of tinea capitis there are single or multiple circular lesions in which the hair is broken off. The hair loss may vary in configuration and the scalp is covered by slightly scaling patches with evidence of hairs piercing the scale, but being snapped off close to the scalp. This condition is caused by superficial fungi which attack the hair shaft itself and the surrounding skin. In the United States, the two most common species of fungi which cause this picture are Microsporum and Trichophyton; the more common varieties include Microsporum audouini and Microsporum canis. These two species generally cause fluorescence of the involved hairs under an ultraviolet light emitting wave lengths of 3650 Angstroms. This may be obtained by using a Wood's light filter. Not all hairs infected with a fungus will fluoresce under this light, but it is certainly a good screening device in checking school children or other members of the family who have been exposed to ringworm. Apparently, the fungus must not only be placed upon the scalp by direct contact or contact with a comb or brush from an infected person, but there must be some injury to the scalp allowing the fungus to gain a foothold. These fungus infections particularly involve children in urban areas where they are in close contact with each other and more commonly involve boys than girls. Uncommonly, these fungus infections of the scalp may call forth a marked reaction called a kerion in which a pustular response develops in a localized area, which often looks like a bacterial infection. This reaction cures itself spontaneously, but may, if the involvement is severe enough, cause scarring and permanent hair loss. In any event, most fungus infections will spontaneously disappear in time if left alone, and will disappear at puberty if the infection is encountered late in childhood. Several uncommonly encountered fungi may cause tinea capitis. They are Trichophyton violaceum, Trichophyton schoenleini and Trichophyton
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tonsurans. The important characteristic of this group is that these fungi do not routinely fluoresce under a Wood's light. Thus, a patient with this type of infection will not be detected if checked only by this method. It is therefore wise to check any patient who has a scaling patch of alopecia by pulling out several of the hairs, looking at them under a microscope directly and culturing them to spot the offending fungus. A positive microscopic diagnosis may be obtained using a 15 per cent solution of sodium hydroxide on several infected hairs. This mixture is warmed slightly, macerating the hairs, and direct examination with the microscope will reveal spores and hyphae. In a differential diagnosis of ringworm of the scalp, one should consider impetigo, alopecia areata and seborrheic dermatitis. Alopecia areata may be differentiated because of the smooth, shiny surface of the scalp, showing no evidence of scaling or inflammation. Seborrheic dermatitis (cradle cap) sometimes causes circular scaling patches on the scalp without hair loss. Impetigo patients usually show heaped-up yellowish crusts on their scalps without evidence of alopecia. Treatment of tinea capitis should consist of griseofulvin tablets given orally in a dosage between 250 and 500 mg. daily, depending upon the weight of the child. Half of this daily dose, 125 to 250 mg., is necessary with ultrafine griseofulvin. These can be taken following the largest meal since fats enhance absorption. Therapy should continue four to six weeks. Shaving the scalp when feasible prevents dissemination to other children and applying adhesive tape across the involved area daily removes infected hairs. In Trichophyton infection, the daily dosage of griseofulvin should be doubled and continued until there is no microscopic evidence of continued infection. (See also Dr. Reiss's article on fungus infections.)
Trichotillomania This is a behavior pattern. Such an individual deliberately pulls the hair out from a given region of the body, either the scalp, eyebrows, eyelashes or body hairs. The exact mental mechanisms causing this behavior are difficult to ascertain, but the individual will pluck the hair out from a given irregularly outlined area. The diagnostic clue will be a scattering of hairs too short to be pulled out, which is in contradistinction to alopecia areata in some patients who may have evidence of hair present but it will be in the form of hairs easily tweezed out. There is usually no evidence of inflammation and the scalp appears completely normal. This type of selfdestruction is not to be taken as lightly as finger sucking and nail biting. It has a more deep-seated prognostic outlook; often psychiatric help may be necessary. These people are not the ones who unconsciously rub or pluck a given area while concentrating or studying. The latter will come in with the hairs sheared off or pulled out in a small region. This is merely a habit, perhaps hard to change, but it is not a difficult psychiatric problem.
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Figure 3. Traumatic marginal alopecia.
Traction Alopecia TRAUMATIC MARGINAL ALOPECIA. This is a form of temporary or permanent hair loss commonly seen in Negroes (Fig. 3). It is produced by excessively tight braiding of the hair24 which, because of continuous pressure on the roots, causes it to fall out, generally around the edges of the pleated area. Often this is reversible if the braiding is discontinued early. In some cases, however, secondary bacterial infection will cause permanent hair loss. PONYTAIL ALOPECIA. Now that ponytail hair styling has become passe, this type of alopecia has become rare. When ponytails were frequently used by women, the constant pressure of the tightly pulled hair often resulted in the appearance of alopecia around the peripheral areas, usually the front and sides of the scalp. This was caused by the same mechanism that produces traumatic marginal alopecia. ROLLER BRUSH ALOPECIA. This is a type of alopecia generally seen along the vertex of the scalp when females use roller brushes to curl their hair. 13 By doing this, they put undue pressure on the hair, and it may be either pulled out in multiple areas along the vertex or snapped off just above the scalp. In all cases, this condition is completely reversible if this type of hair curling is discontinued. However, these brush rollers may stick into the scalp to cause a low grade folliculitis, which may complicate the diagnosis. Pressure Alopecia This is a type of alopecia originally described by Abel and Lewis. l They reported eight cases that appeared following protracted surgical procedures in which the head was held in one position. This type of alopecia usually appears several days after the operation in the form of a soft, moist
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area on the posterior aspect of the scalp generally involving no more than a silver dollar sized area j it looks like localized folliculitis. Many times this initial stage of the alopecia is missed since the patient, having undergone extensive surgery, is under considerable sedation. It first resembles alopecia areata, but later usually takes on an irregular outline showing snapped off hairs at the periphery. There may be some hyperpigmentation at the time the patient first consults the physician since the original dermatitis has subsided. An excellent prognosis may be offered since hair completely returns without therapy.
Postfuruncular Alopecia Postfuruncular alopecia3 follows the development of single or multiple furuncles in the scalp. The alopecia is, in this case, confined to the region of the elevation caused by the furuncle. The hair loss begins shortly after the infectious process starts and the prognosis is excellent. It is caused by pressure from below upon the pilosebaceous structure causing hair to fall out. No therapy is required.
Tick-Bite Alopecia In several western states, it has been reported that tick bites produce inflammatory dermatitis,21 which may subsequently affect the deeper scalp structures causing a transient localized area of alopecia. This resembles alopecia areata or the "moth-eaten" alopecia of secondary syphilis when there has been more than one tick bite.
Figure 4.
Alopecia mucinosa with follicular spines in the areas of alopecia.
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Alopecia Mucinosa This rare disease generally arises with an area of alopecia some place on the scalp-but not always (Fig. 4).19 If it arises in the scalp, there are follicular papules in the center of an area of baldness. The hair loss is primarily due to mucinous changes in epithelial cells of the pilosebaceous follicle with secondary loss of hair. It is now believed that this mucinous degeneration in the hair follicles may accompany several basic conditions, one of which may be self-limited and may spontaneously regress. The condition may also be seen in other inflammatory lesions of the scalp and sometimes in mycosis fungoides. In the latter condition, the lesions on the scalp will respond to x-radiation, but will recur. More investigation is necessary on the pathophysiology of this condition.
LOCALIZED SCARRING ALOPECIA
Discoid Lupus Erythematosus Clinically, scalp lesions generally appear as areas of alopecia showing some redness of the bald area in their initial stages and a minimal amount of scaling and follicular plugging. The alopecia is permanent and healing takes place with scarring and hypo- or hyperpigmentation. Old· healed areas of lupus erythematosus showing atrophy may be adjacent to areas of activity. Usually, one is not confronted with a diagnosis of only lesions on the scalp i often other lesions appear on the light-exposed portions of the trunk, hands and ears. In Negroes, older areas may exhibit white, atrophic, depigmented areas with a hyperpigmented festooned border in which the active process is still present. Biopsy, generally of the active area on the scalp, usually leads to proper diagnosis. Frequently, the problem of differentiating discoid lupus erythematosus from acute disseminated lupus erythematosus is difficult. Other dermatoses resembling this are seborrheic dermatitis, polymorphous light sensitivity and occasionally psoriasis. Therapy for discoid lupus erythematosus of the scalp is the same as elsewhere on the body. The antimalarial drugs (chloroquine tablets) may be prescribed. They should be given in a dosage of 250 mg. once or twice a day for three to four weeks. Continuous therapy with high doses for long periods should be avoided because damage to the cornea and retina have been noted. With some of the newer steroids, topical and sublesional therapy has proved effective when they are injected 3 mm. below the surface of the skin. Triamcinolone acetonide, 0.1 cc. in each centimeter of skin showing lupus erythematosus, has proved helpful. The application of the same agents topically under occlusive therapy (using polyethylene wrap or Blenderm tape) often leads to a resolution where in the past other topical modalities were not effective.
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Scleroderma Scleroderma, when it involves the scalp and causes alopecia, generally takes one of two forms. The first is a linear midline lesion extending onto the forehead. It causes complete absence of hair in the involved area, resulting in the classical coup de sabre appearance. Most often this begins in the first decade of life. The area shows a sclerotic patch which is bound down to underlying structures. There is no hair growing in the area, which is depressed below the skin level. This may be associated with facial hemiatrophy. The second variety is morphea (localized scleroderma) which appears as an edematous patch surrounded by a violaceous halo. As time passes, the area loses its acute activity and becomes bound down with the classical ivory color seen in morphea. Most patches involute spontaneously, leaving permanent baldness. No therapy for either condition is known to be effective.
Pseudopelade This is a rare, scarring, progressive alopecia which appears as an area of atrophy (Fig. 5). Upon inspection, no activity is seen around edges of the areas of alopecia to account for the hair loss. The condition usually begins as a small area on the top of the head near the vertex which slowly progresses giving an irregular outline to the alopecia. It looks like "footprints in the snow." The involved area shows thinning skin and a loss of subcutaneous scalp tissue so that the area is depressed below the skin level. This and other scarring alopecias may, when the disease process has burned out, take on this "footprint" appearance. Lupus erythematosus, folliculitis decalvans and lichen planopilaris must be considered in the diagnosis. Therapy, when scarring becomes too noticeable, consists of grafting other scalp hair into the area of alopecia.
Figure 5. Pseudopelade showing the irregular outline of alopecia, scarred surface, and no inflammation around the hair follicles at the periphery.
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Alopecia due to excess ionizing radiation.
Lichen Planopilaris This is an unusual and rare variety of lichen planus which produces small elevated papules about the hair follicle. It may involve the scalp, resulting in irregularly outlined areas of alopecia with central atrophic whitish scars. There may be similar small grouped papules on the body giving a clue to the diagnosis. This condition is different from folliculitis decalvans and pseudopelade of the scalp, but they all eventuate into similar scarring alopecia. No therapy is effective.
Injury, Burns, and Ionizing Radiation Injuries to the scalp deep enough to avulse the entire hair-bearing area if not replaced adequately, or if there is not enough tissue to cover the defect, will heal with scarring leading to irregularly outlined areas of alopecia. Burns which destroy the full thickness of skin lead to permanent scarring and alopecia. X-ray burns causing permanent alopecia and scarring may develop if more than 1200 roentgens of superficial x-ray are given to anyone region of the scalp (Fig. 6). Broad telangiectasias course across the area of baldness. The hair loss is usually not complete because less radiation reaches the periphery of the scalp.
Bacterial Infection, Furuncles, Carbuncles and Acne Keloidalis Furuncles and carbuncles of the scalp, especially those localized in the suboccipital region, will heal with irregular scars without hair. In some individuals, especially Negroes, there may be an excessive amount of scar tissue resulting in acne keloidalis. This causes scarring in the nuchal region with erythematous follicular keloids elevated above skin level, leading to permanent alopecia in the area involved. Therapy includes, in these cases, the production of adequate drainage of the lesion and systemic administration of appropriate antibiotics. Acne keloidalis may require dry ice application to flatten excessive scar tissue. Intralesional injection
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of corticosteroids may also be effective when given in a fashion similar to the treatment noted under alopecia areata.
Minor Ectodermal Defects Occasionally one may see a newborn infant with a small, shiny erythematous patch across the parietal area of the scalp} It may range in size up to a diameter of 3 cm., and there is no hair present. These defects are often believed to be caused by the use of instruments at the time of delivery, but in reality they are small ectodermal defects. As time passes, the area becomes depressed below skin level and .shows no tendency for hair growth. If one sees a minor ectodermal defect, it is wise to be certain there are no other ectodermal malformations in the child. No therapy is necessary because the adjacent hair usually covers the defect.
Primary and Metastatic Neoplasia of the Scalp Basal cell carcinomas have at times caused areas of alopecia usually beginning on the light-exposed areas of the forehead or on the bald pate. The carcinoma gradually enlarges through the years, forming an active rolled border with complete destruction of the hair roots and leading to baldness in the involved regions. These are nonmetastasizing tumors and can usually be eradicated either by surgery or x-ray. Metastatic carcinoma may also involve the scalp and cause alopecia;5, 20 by far the most common site of the primary tumor is the breast. The affected scalp takes on a fairly characteristic appearance in which there is a slowly progressing area of alopecia showing a cobblestone appearance. Occasionally, this may be mistaken for alopecia areata, or the bound-down appearance of morphea (localized scleroderma). A history of previous surgery on a malignant breast tumor can be
Figure 7. Folliculitis decalvans with its characteristic infected hair follicles.
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helpful in making the diagnosis, but there have been reports of the metastasis appearing in the scalp before the primary nodule was discovered in the breast.
Folliculitis Decalvans This is also a rare form of scarring alopecia caused by the production of small follicular pustules leading to destruction of the pilosebaceous apparatus (Fig. 7). Usually, activity begins in a small area with central atrophy and then spreads in a centrifugal fashion so that the folliculitis is seen at the periphery of the lesion. The outline is irregular and not every hair in the patch of alopecia may be affected. Therapy in this condition should include adequate systemic antibiotics and topical antibiotics under occlusive polyethylene wrap therapy. The end result without treatment or with unsuccessful treatment is that of irregularly outlined areas of alopecia with smooth, depressed scarring in the center of the lesion.
DIFFUSE NONSCARRING ALOPECIA
Male Pattern Hair Loss
This is the usual hair loss seen in young males. It generally begins in the early twenties when there is an elevation of the anterior hair line forming the classic "widow's peak." A genetic predisposition plus the development of a normal amount of androgenic hormones are essential for its progression. It has long been known that people who are castrated before the age of puberty, no matter what their genetic predisposition, do not develop this type of alopecia. It is interesting that androgenic stimulation tends to cause an increase in growth of the hairs on the rest of the body. It is paradoxical that only hair follicles on top of the scalp seem to have genetic predisposition for hair loss. Persons who suffer from male pattern hair loss may also suffer from dandruff. It has been proposed, but not proved, that the unsaturated acids, squalene and oleic acids, found in sebum may have some effect in causing increased hair loss in both sexes. 7 All the other causes suggested for this type of hair loss, i.e., decreased blood supply, 6 tight scalp, and a lack of subcutaneous fat on the scalp, have been disproved. Therapy is unsuccessful. Female Hair Loss or Thinning
In the past few years there have been increasing complaints of hair thinning in women. 4 , 25 Studies have failed to reveal any true cause for this particular type of thinning which has much the same pattern as that of the male who is progressing to complete baldness across the vertex of the scalp. Women usually have thinning only in comparable areas and generally it does not progress to severe alopecia. Nevertheless, this problem has been increasingly perplexing to the dermatologist; anyone confronted with it learns that the loss of woman's crowning glory throws fear into their hearts. Many theories as to the cause of this condition have been put forth, including the idea of it being due to hair dyes, permanent waves,
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teasing, wave sets, or hair sprays. Nylon brushes sometimes cause a snapping off of the hairs and produce thinning. Extensive endocrinological work-up of the pituitary, thyroid, adrenal and ovary has failed to reveal any cause. Some question has been raised as to whether there is a true increased incidence in this condition or merely an increased awareness, since the female is now more devoted to the adoration of her hair.26 It may be that sometime during the adult life of the female, her normal hair count undergoes a decrease, establishing a new norm. When this occurs, she is emotionally disturbed out of all proportion to the hair loss. Elderly women occasionally will have extensive thinning across the vertex, but generally by that time they are not worried about their physical appearance. Many times the normal hair fall of between 40 to 90 hairs a day will have been re-established, yet the individual is counting every hair as it comes out and worrying. In most cases, extensive reassurance of the patient is all that is required. Treatment is directed at ascertaining that the patient is not causing, through some manipulation, the snapping off of her hair at varying lengths above her scalp either by using hair straighteners or curling rollers, or by performing some other traumatic manipulation. These can produce hair thinning that is merely the snapping off of the distal tips. Also, an endocrinological work-up, although rarely rewarding, should be done.
Total and Universal Alopecia Alopecia totalis may be the end result of alopecia areata (Fig. 1, B). In 20 per cent of 736 cases at the Mayo Clinic,!" five or more years elapsed before development of complete hair loss. Ordinarily the loss progresses to a total loss at a slower rate in children than in adults, but its occurrence is more frequent. Multiple areas of alopecia, involving hairy areas other than the scalp, portends to a more severe and chronic form with an increased likelihood of progression to total alopecia. In the totalis group, 20 per cent of the children and 30 per cent of the adults in the Mayo series showed no significant regrowth. Only about 1 per cent of the children and 10 per cent of the adults showed permanent regrowth. It should be emphasized that approximately 40 per cent of the children and 30 per cent of the adults who developed total alopecia had subsequent periods of normal or near normal hair growth. In these groups of patients with alopecia totalis or universal alopecias there is an increased incidence of cataracts. These cataracts were seen in 7 of the 736 cases at the Mayo Clinic. l5 These patients were mainly in their fourth and fifth decades and had a posterior subcapsular type of cataract.
Alopecia of the Newborn A large number of newborn infants between one and four months of age have a bald area, most often across the posterior aspect of the scalp.
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This is believed to be caused by frictional rubbing of the scalp against the bed or cradle, but this is not usually true since most infants lose their first growth of hair completely during the first six months of life. 12 If this hair loss, which takes place as a normal physiological mechanism, occurs in sharp waves, there may be complaints from mothers that the child has developed alopecia. For the most part, this goes unnoticed because there is already some asynchronism of each hair follicle. This type of hair loss is generally unnoticeable by the time the child is six months old. Drug-Induced Alopecia
The following drugs have been shown to cause alopecia: nitrogen mustard, heparin and other anticoagulants, thallium and all the anticancer drugs, particularly the folic acid antagonists. Several pathological mechanisms account for drug-induced hair loss. In heparin alopecias there is a sudden increased number of resting hairs (telogen stage), so that a latent period of from one to four months is necessary before the alopecia develops. Since this type of hair loss may be phasic, and each follicle is in a different phase, it is understandable why many physicians never notice it. This same mechanism holds true with all anticoagulant drugs. With cancericidal drugs another mechanism prevailsY Here growing hair (anagen stage) is lost. Depending upon the dose of the cancericidal drug, the entire activity of hair growth may be suspended. If the insult is sufficient, as much as 80 or 90 per cent of the hairs are lost in a wave-like fashion, generally beginning with a short latent period of one to three weeks. If the insult is not sufficient, the hair will not be lost but will merely show a narrowing hair shaft. This likewise happens with nitrogen mustard, thallium, and folic acid antagonists. Postfebrile Hair Loss
Only occasionally in this era of antibiotics do individuals run a high enough temperature to cause hair fall. It may follow any febrile illness, usually pneumonia, in which the temperature is elevated above 103° F. for a long period.This temperature causes an increased number of the hairs to progress to the resting stage (telogen phase) and they usually begin to be shed two to three months after the illness, with most of the shedding being completed in six to eight weeks. There is no area of localization of the alopecia and usually all of the hair will regrow. Therapy should consist of reassuring the patient that all of the hair will return in time. Secondary Syphilis
It has long been known that syphilis, in its secondary stages, may cause an alopecia in which there is hair loss in individual areas which are
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generally spread across the scalp, but classically are located around the sides of the head. The clinical appearance is "nlOth-caten." The clue to this diagnosis is the presence of other manifestations of secondary syphilis such as a generalized maculopapular eruption. The quantitative serum test for syphilis (STS) is always elevated. Once the individual has been treated with adequate amounts of penicillin, there is complete restitution of this hair and no permanent alopecia. Postpartum Hair Loss
Most women know that many members of their sex complain of increased hair loss following childbirth, only to have it return to normal. Lynchfield followed a group of normal women through pregnancy and found that they developed an increased percentage of resting hairs which eventually fell out about two or three months following delivery. There was noticeable thinning in four of the 26 patients and he noted that roughly 40 to 50 per cent of the hairs must be lost before visible thinning is noted on the scalp. There is some good evidence that during the second and third trimesters of pregnancy fewer hair follicles enter into the resting stage. As a consequence, anywhere from two to three months postpartum there is an accelerated increase in resting hairs which are subsequently lost. Thus, as previously stated, even though there lllay be a complaint by the woman who is pregnant that she is losing her hair and it seems to be somewhat thinner, there is no sound evidence pointing to any significant regions of alopecia. Schiff and Kern found hair loss usually began by the tenth to thirteenth week postpartum and the majority of the women in their series had noted their hair fall by the twentieth week. 23 It was also noted that most of these persons had complete restitution of hair after several months. There is weighty evidence for a hormonal factor in the causation of postpartum hair loss, the most pressing one being that if women who had had multiple pregnancies and suffered from repeated postpartum hair loss became pregnant again before the tenth to thirteenth week, they did not subsequently develop postpartum alopecia. The prognosis is excellent for complete restitution of all of the hair that was lost postpartum. Congenital Alopecias
Congenital alopecia is the absence of hair or development of extremely poor hair on the scalp or on the rest of the body (Fig. 8). The infant may never have any hair, or the first hair may develop normally only to fall out. Subsequent growth of hair is shorter, lighter, and the hairs are fewer in number than normal. Most of these people have normal teeth and nails and enjoy good health. There are apparently two modes of transmission of this characteristic:
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Figure 8. Congenital alopecia in 2 generations.
in some families it is transmitted as a dominant gene and in others as a recessant gene. No therapy is of any avail. Monilethrix is a rare condition in which the hair shaft has alternate normal areas and thinned areas so that the hair appears beaded (Fig. 9). As a result, the hairs tend to snap off before they reach normal length. The appearance of the scalp is one of a crewcut or of a generalized diffuse hair loss. The condition is most likely to appear on the posterior aspects of the scalp, but may involve any area of the body. It is usually associated with follicular hyperkeratotic papules in the involved areas and on adjacent areas of the neck and upper extremities. This condition may arise at any time in life. Usually it begins prior to the first year, but may begin at any time-particularly at puberty. The diagnosis is simple, once one suspects it, in that a microscopic examination of the hair will alternately show a zone of thin shafts next to a zone of normal hair shafts. This has been reported as a dominant hereditary condition since several members of the same family may have it; others report it as a completely recessive trait. Monilethrix must be differentiated from pili annulati or "ringed hairs." This condition shows alternate light and dark bands in the hair with no alteration in the circumference. This phenomena is caused by air in the matrix of the hair in light areas. Monilethrix must also be differentiated from trichorrhexis nodosa. This
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Figure 9. Monilethrix. Congenital beading of the hair leads to its easy fracture so that it appears the hair has been cut short.
condition has a tendency to fracture the hair shaft so that there are tuftlike breaks in the shaft of the hair, causing it to snap off more easily. The usual causes are excessive manipulation of the hair, excessive brushing, or some sort of mechanical or beautifying destruction of the normalconfiguration of the hair shaft. There is no therapy for the first two conditions. In the trichorrhexis nodosa, extreme caution should be exercised in the way the hair is coiffured. DIFFUSE SCARRING ALOPECIA PERIFOLLICULITIS CAPITIS ABSCEDENS ET SUFFODIENS (Dissecting Folliculitis of the Scalp). Some individuals develop perifolliculitis of the scalp. Multiple draining sinuses generally involve a large portion of the scalp so that the entire area is one mass of necrotic draining lesions interconnecting to form a boggy mass across the area of the scalp. Many of the patients have comedones (blackheads) interspersed in each of the hair follicles, often associated with acne elsewhere on the body. Therapy should consist of the intensive use of external and internal antibiotics. When an extended area of the scalp is involved, temporary x-ray epilation of the scalp may at times be necessary to arrest this troublesome process when all other methods have failed. It is evident that those conditions discussed under localized scarring alopecia may in the severe case cause a diffuse scarring alopecia.
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REFERENCES 1. Abel, R. R. and Lewis, G. M.: Postoperative (pressure) alopecia. A.M.A. Arch. Dermat. 81: 34-41, 1960. 2. Andrews, G. and Domonkos, A.: Diseases of the Skin. Philadelphia, W. B. Saunders Co., 1963. 3. Butterworth, T. and Fowler, J. C.: Postfuruncular alopecia. A.M.A. Arch. Dermat. 80: 570-573, 1959. 4. Butterworth, T. and Strean, L. P.: Clinical Genodermatology. Baltimore, Md., Williams & Williams Co., 1962. 5. Cohen, I., Levy, E. and Schreiber, H.: Alopecia neoplastica due to breast carcinoma. Arch. Dermat. 84: 490-492, 1961. 6. Cormia, F. E. and Ernyey, A.: Circulatory changes in alopecia. Arch. Dermat. 84: 772-789, 1961. 7. Flesch, P.: Hair loss from sebum. A.M.A. Arch. Dermat. 66: 1-9, 1953. 8. Gentles, J. C. and Barnes, B. A.: A report on animal experiments with griseofulvin. A.M.A. Arch. Dermat. 81: 703-708, 1960. 9. Guy, W. B. and Edmundson, W. F.: Diffuse cyclic hair loss in women. A.M.A. Arch. Dermat. 81: 205-207, 1960. 10. Haynes, H. A., Jr. and Parry, T. L.: Alopecia areata associated with refractive errors. Arch. Dermat. & Syph. 59: 340-343, 1949. 11. Kligman, A. M.: The human hair cycle. J. Invest. Dermat. 33: 307,1957. 12. Kligman, A. M.: Pathological dynamics of human hair loss. Arch. Dermat. 83: 175, 1961. 13. Lipnik, M. L.: Traumatic alopecia from brush rollers. Arch. Dermat. 84: 493-495, 1961. 14. Montagna, W.: The Structure and Function of Skin. New York, Academic Press, Inc., 1958. 15. Muller, S. A. and Winkelmann, R. K.: Alopecia areata-an evaluation of 736 patients. Arch. Dermat. 88: 290-297, 1963. 16. Orentreich, N., Sturm, H. M., Weidman, A. I. and Pelzig, A.: Local injection of steroids and hair regrowth in alopecias. A.M.A. Arch. Dermat. 82: 894-902, 1960. 17. Pecoraro, V., Astore-Ignacio and Barman, J. M.: Cycle of the scalp hair of a newborn child. J. Invest. Dermat. 43: 145, 1964. 18. Pillsbury, D. M., Shelley, W. B. and Kligman, A. M.: Dermatology. Philadelphia, W. B. Saunders Co., 1956. 19. Pinkus, H.: Alopecia mucinosa. A.M.A. Arch. Dermat. 76: 419-426,1957. 20. Ronchese, F.: Alopecia due to metastases from adenocarcinoma of the breast. Arch. Dermat. & Syph. 59: 329-332, 1949. 21. Ross, M. S. and Friede, H.: Alopecia due to tick bite. Arch. Dermat. 87: 609-611, 1963. 22. Savill, A. and Warren, C.: The Hair and Scalp. Baltimore, Md., Williams & Williams Co., 1962. 23. Schiff, B. L. and Kern, A. B.: Postpartum alopecia. Arch. Dermat. 87: 609-611, 1963. 24. Slepyan, A. H.: Traction alopecia. A.M.A. Arch. Dermat. 78: 395-398, 1958. 25. Smith, M. A. and Wells, R. S.: Male-type alopecia, alopecia areata, and normal hair in women. Arch. Dermat. 89: 95-98, 1964. 26. Sulzberger, M. B., Witten, V. H. and Kopf, A. W.: Diffuse alopecia iI'l women. A.M.A. Arch. Dermat. 81: 556-560,1960. 27. Van Scott, E. J., Reinertson, R. P. and Steinmuller, R.: The growing hair roots of the human scalp and morphologic changes therein following Amethopterin therapy. J. Invest. Dermat. 29: 197, 1957. 28. Weidman, A. I., Zion, L. S. and Mamelok, A. E.: Alopecia areata occurring simultaneously in identical twins. A.M.A. Arch. Dermat. 76: 424-425, 1956. 29. Winkelmann, R. K. and Jaffe, M. 0.: Nerve network of the hair follicle in alopecia areata. A.M.A. Arch. Dermat. 82: 750-753, 1960.
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