Chapter 126: Hair disorders

The hair of our scalp is referred to as our crowning glory and the threat of hair loss in both sexes provokes extraordinary anxiety bordering on grief in some people. It behoves us as medical practitioners to treat the patient presenting with ‘I’m losing my hair’ with appropriate support and understanding. Likewise, the problem of hirsutism provokes similar anxiety and concerns about body image. Interestingly, women present with hair loss more than men.

An understanding of the process of normal hair growth is necessary to comprehend and evaluate hair disorders. Each follicle progresses quite independently through regular cycles of growth and shedding (see FIG. 126.1).

The three phases of follicular activity are:³

1. Anagen phase—the active growth phase of hair production.

   • The dermal papilla stimulates division of epithelial cells that produce the hair shaft.

   • The hair shaft grows 1 cm per month.

   • It lasts for about 2.5–5 years on the scalp (average 1000 days).

   • It lasts 1–2 months on eyebrows and eyelashes and 6–9 months in the axilla and pubis.

   • It varies between individuals.

2. Catagen phase—a short transition phase from active growth to inactivity (the involutional stage).

   • The base of the hair becomes club-shaped.

   • It lasts for only about 2 weeks.

3. Telogen phase—the resting (dormant) phase of the cycle at the end of which the club hair with its non-pigmented bulb is shed.

   • This lasts 2–4 months (average 100 days).

   • The percentage of hairs in telogen varies from site to site—10% in scalp to 60–80% in pubic hair.¹

   • The hair is anchored in the follicle but does not grow longer.

   • The follicle then re-enters anagen.

Thus every 3–5 years every hair on the scalp is shed and replaced.

Some facts on hair numbers³

• Hair growth is asynchronous (i.e. continuous production and shedding).

• Humans produce 1 km of hair a month.

• About 50–100 hairs are shed daily without a reduction in density.

• The scalp contains, on average, 100 000 hair follicles.

• The hair follicle is subject to melanocytic activity.

• At least 25% of hair must be shed before a noticeable loss of density occurs.

• Hair loss counts consistently above 100 per day indicate excessive hair loss.

• Significant hair loss tends to block the shower drain or be visible all over the pillow.

Key history

• Onset, duration, quantity and rate of loss

• Localised or generalised loss

• Associated symptoms (e.g. pruritus, scaling, pustules)

• Systems review including fever, acute illness, surgery, stressors

• Symptoms of hyperandrogenism in women (e.g. hirsutism, acne)

• Endocrine features

• Past history including skin disorders, cancer, thyroid disorders

• Family history of hair loss

Key examination

• General review with emphasis on endocrine system and examination of scalp

• Look for exclamation mark hair, ‘white bulb’ hair, state of bald patch (clean, scaly, scarred or inflamed) and the unusual pattern of trichotillomania

Key investigations

Consider:

• FBE/ESR

• pituitary hormones (FSH/LH/prolactin)

• hair pull test

• trichogram

• scalp biopsy

• skin scrapings and hair sample for fungal microscopy and culture

Alopecia areata, alopecia totalis and alopecia universalis

Alopecia areata is a disorder of the hair follicle causing a sudden onset of localised or diffuse hair loss. It is thought to be an autoimmune disorder that has a genetic susceptibility (20% have a positive family history). Men and women are affected equally, with the disorder most commonly occurring in the first two decades of life.⁴ The hairs are affected during the growth phase, resulting in cessation of anagen.

Clinical features

• Complete hair loss (small patch or diffuse)

• Pigmented hairs often lost first

• Clean normal scalp

• No or minimal inflammation

• Exclamation-mark hairs, especially around the periphery (see FIG. 126.2)

Associations

• Loss of facial (beard in men) or body hair

• Nail changes—dystrophy or pitting

Alopecia areata²

An unpredictable pattern of remission and relapse is characteristic. Patches may:

• regrow hair spontaneously (up to 50% within 12 months if a single patch)

• stay the same for many months

• enlarge and coalesce with other patches

The prognosis is poorer with more widespread involvement and younger age of onset (<5 years of age).

Localised patches

Treatment options include:

• topical potent corticosteroid (especially in children), e.g. betamethasone dipropionate 0.05% cream or lotion, once or twice daily for 3–4 months

• intralesional steroids—triamcinolone 10 mg/mL or betamethasone acetate/phosphate 5.7 mg/mL bd (caution needed on face—risk of cutaneous atrophy). Multiple injections required.

• topical minoxidil 5% bd (for 4 or more months) only when hair growing

Extensive area (>50% loss)

Treatment options include:

• Counselling

• Alopecia areata support group

• Use of cosmetic aids (e.g. eyebrow tattoos, wigs, hair pieces, scarves) and camouflage

• Topical steroids not recommended—ineffective

• Topical immunotherapy or systemic immunosuppresion under specialist care may help

• Systemic steroids only for active and progressive cases: prednisolone 0.75 mg/kg (o) daily for 3–4 weeks, then taper off over 2 months

Alopecia totalis

• This is where the alopecia extends over the total scalp.

• There is, at best, a 50% chance of hair recovery in a fit adult but only a very small chance in childhood.

Alopecia universalis

• This is where the alopecia involves the eyebrows and eyelashes as well. Recovery is rare.

Scarring alopecia³

In this condition, the hair follicles are damaged and if the follicular openings cannot be seen with a magnifying lens, regrowth of hair cannot be expected. Thus, the process is irreversible and a scalp biopsy is essential to determine the diagnosis. Apart from obvious causes such as trauma, severe burns, a carbuncle and scalp ringworm with kerion, the causes of scarring alopecia are as follows.

• Lichen planopilaris—this is a variant of lichen planus that produces small follicular papules in the scalp that tend to heal with scarring and destruction of hair follicles; treatment, which is difficult, includes corticosteroids, antimalarials and etretinate³

• Discoid lupus erythematosus—this gives a similar picture to the former and tends to be treated in a similar manner

• Folliculitis decalvans—a chronic folliculitis of the scalp, probably as a response to staphylococci on the scalp; treated with long-term tetracyclines

• Pseudopelade—a slowly progressive, non-inflammatory, scarring condition causing patchy areas of hair loss without any obvious preceding skin disease

Telogen effluvium⁵

Telogen effluvium, which is increased shedding of hairs in the telogen phase, is one of the most common causes of diffuse hair loss and can be triggered by a variety of stressors.

It is worth noting that follicular matrix cells have a high metabolic rate second only to haematological tissue and stress can result in shunting into premature telogen with cessation of anagen.³

An obligatory delay occurs between the ‘insult’ or precipitating event and the onset of hair shedding because the hair follicle cycles through catagen and telogen—approximately 2–3 months when the club hairs with white bulbs of telogen are shed.

Greater than 25% of hair must be lost before there is a perceptible thinning and in this disorder up to 50% loss is common.

Patients usually complain of large clumps of hairs with white bulbs coming out with gentle tugging on combing or shampooing (this can exceed 150 hairs per day compared with the normal average of 50–100 hairs).

Stress precipitants of acute telogen effluvium

• Any severe stress

• Childbirth (common)

• High fever

• Weight loss, especially crash dieting

• Trauma—surgical or accidental

• Oral contraceptive pill (OCP) cessation

• Perimenopause

• Malnutrition

• Haemorrhage

Course of telogen effluvium

If uncomplicated, spontaneous recovery can be expected in 6–9 months so reassurance with explanation is usually all that is required. If it persists longer than 6 months, consider the chronic idiopathic form or an unmasked androgenetic alopecia. However, if there is concern about non-recovery and the stress factors are corrected, topical minoxidil 5% lotion (2% if too irritant) twice daily for a minimum of 4 months is an option.² Referral to a specialist is advised for relapsing episodes or incomplete recovery.

Chronic telogen effluvium⁶

This occurs usually in perimenopausal and postmenopausal women. It may be primary and idiopathic or secondary to hypothyroidism, hyperthyroidism, malnutrition or cancer. The feature is episodes of dramatic hair shedding that recover but recur weeks to months later and last up to several days. It does not result in obvious balding—it is self-limiting and does not usually need treatment.

Anagen effluvium

This is hair loss during the anagen phase and is typically seen in association with cancer chemotherapy and radiotherapy to the scalp, which results in immediate metabolic arrest. Hair loss is diffuse, involving the whole scalp. Anagen hair shafts are identified by their long and pigmented hair bulb. The follicle may remain in anagen, leading to a quick recovery, or move into telogen, thus delaying growth by about 3 months.

Drug-induced alopecia

Drugs are a very important cause of alopecia (see TABLE 126.3). They may cause telogen effluvium, anagen effluvium or accelerate androgenetic alopecia.

Drugs tend to cause telogen effluvium but cancer chemotherapy, radiation to the scalp, thallium/mercury/arsenic and colchicine in high dosage cause anagen effluvium. Acceleration of androgenetic alopecia is caused by hormone therapy, namely the OCP, danazol, testosterone and anabolic steroids.

Androgenetic alopecia (male pattern baldness)

This is the most common form of alopecia, which is age related and is genetically determined in addition to being androgen dependent. The key androgen is dihydrotestosterone, which is produced from testosterone by 5α reductase. The typical pattern of hair loss is recession of the frontotemporal hair with progression to the crown, with some men losing hair quickly and others more slowly. In others the pattern is unpredictable. The typical male pattern is shown in FIGURE 126.3.

Alopecia affects 30% of men by age 30 and 50% by age 50.

Androgenetic alopecia in women

• In women, the pattern of hair loss is different from men.

• Diffuse thinning occurs, usually on the top of the head (the crown). The front hairline usually remains but in some women this can recede with bitemporal loss (see FIG. 126.4).

• Although hair loss can appear in men and women as early as the 20s, it may not appear before the age of 50 in women.

• Some women notice a short period of considerable hair loss but this may be followed by a long stable period of no loss. Total loss of hair rarely occurs in women.

• It may be unmasked after an episode of diffuse hair loss such as after childbirth or an acute illness.

• Hair loss may be accelerated by conditions associated with androgen excess (e.g. polycystic ovarian syndrome), however, fewer than 5% of women with androgenic alopecia will have elevated serum androgens.²

Treatment (men)

It is appropriate to counsel men to accept their balding as part of a natural ageing process. This includes cutting the hair short to make it look better cosmetically. If baldness is not acceptable, some options include wearing a toupee, a wig or other hair substitute or having a hair transplant operation. However, with hair transplantation, the new hair is often just as likely to disappear as the original hair.

Medical treatments are generally expensive and improvements are only sustained for the duration of treatment, so it is important to explore the patient’s expectations when discussing treatment options.

Available medications²

• Minoxidil 5%, 1 mL applied once or twice daily to the scalp for at least 6–12 months. The results vary from good to no change. Reassure the patient that it is normal for hair shedding to increase in the first 4–6 weeks of minoxidil treatment.

• Finasteride 1 mg tablet taken daily for a minimum of 2 years. There is a risk of sexual dysfunction (e.g. erectile dysfunction, reduced libido) and gynaecomastia (rare), which tend to resolve with time or cessation. Rare reports of persistent sexual dysfunction after stopping finasteride have been published. Mental health problems have also been noted.

• For severe cases, use topical minoxidil and oral finasteride as combination therapy.

Treatment (women)

Physical treatments/hair styling

This includes the use of wigs, hair transplantation and camouflage. Wigs can be worn on the whole head or on the bald spot, or fibres can be interwoven into the remaining hairs.

Camouflage can be used either by having the existing hair bleached by a skilled hairdresser or by colouring the scalp the same colour as the hair. Mascara can be lightly brushed into the roots of the hair at receding hairlines or along gaps.

Medications¹

• minoxidil 5% topical application bd for at least 6–12 months (to assess efficacy) although one large trial has approved its effectiveness generally in women⁷

• or

• spironolactone 50 to 100 mg daily, increasing to 200 mg daily if no apparent benefit after 6 months; monitor blood pressure, kidney function and liver biochemistry

Spironolactone is contraindicated in pregnancy and side effects include postural hypotension, polyuria and irregular menses. Concurrent use with the combined oral contraceptive pill is appropriate to control menses and for contraception in women of reproductive age.

As for men, these drugs tend to prevent further loss and, if effective, need long-term use.

Trichotillomania (hair pulling)

This is patchy hair loss caused by deliberate plucking or twisting of hair shafts. It is reasonably common in young children, where it may be of little significance, simply being a ‘habit’. In older children and adults it may be an obsessive compulsive disorder often associated with stress.³

Clinical features

• Incomplete patchy alopecia

• Hairs of different length

• Hairs broken and twisted

• Strange pattern of loss

• Tends to occur on side of dominant hand

• Eyelashes or eyebrows may be involved

Loose anagen (growing hair) syndrome⁸

This is a disorder of the hair follicle characterised by the ability to pluck anagen hairs painlessly from the scalp by gentle pulling. It presents as very thin, wispy new hair growth. It is an autosomal dominant trait.

Clinical features

• Thin wispy hair with tatty ends

• More common in girls; can affect boys

• Onset in early childhood—usually <5 years

• Large clumps easily pulled out at play

• Light microscopy of hair shafts aids diagnosis

Outcome

• Spontaneous improvement with age

• Usually normal hair by teens

Treatment

• Reassurance and explanation

• Gentle hair care

Traction alopecia

Traction alopecia is thinning of the hair seen in female children and young women due to very tight hairstyles, as with ponytails, excessive hair rolling and hair braiding in particular. The bald areas that show short broken hairs and sometimes scarring are found in areas of maximal tug. The most common type is ‘marginal alopecia’ found in the forehead and sides at the hair edges (see FIG. 126.5). Patients should be advised to cease the procedure. Rollers that cause traction can be replaced by those that heat.

Trichotillomania⁸

This occurs in children typically between 4 and 10 years, usually as a nocturnal habit; parents may be unaware of the hair pulling. The affected areas are usually on the anterior and temporal areas of the head (see FIG. 126.6). The areas are never completely bald. A characteristic feature is an irregular-shaped area of incomplete patchy alopecia containing hairs of different length. The variable length is due to the fact that some hairs will not break with pulling while others will break at varying distances from the scalp surface. There may be associated follicular pustules.

However, scrapings should be taken to exclude a particular type of tinea capitis (black dot ringworm) caused by Trichophyton tonsurans. The management is similar to thumb sucking or nail biting with a low-key approach. It does not imply a significant psychological problem.

Localised alopecia areata

This can also occur in children. Most childhood cases resolve spontaneously but it can progress to total hair loss or recurrent alopecia can occur. Regrowth decades later is a possibility. Treatment with potent topical steroids for 12 weeks or so may help.

Tinea capitis

This is a dermatophyte infection that produces an area of incomplete, ‘unclean’ alopecia with various degrees of scaling and inflammation of the scalp surface. A boggy swelling (kerion) can develop in severe cases. Important dermatophyte carriers include pet puppies, kittens and rabbits. Wood’s light examination will be positive in only 50% of cases. Confirm diagnosis with scalp scrapings for microscopy and culture.

The following procedures can be useful in determining diagnosis and prognosis.

The hair-pull test³

In this simple method, 50–100 hairs are grasped between thumb and forefinger and gently pulled proximal to distal. This is repeated 6–8 times and should yield a total of 2–5 telogen hairs, which can be analysed. More than eight hairs per pull is abnormal.

Trichogram

Twenty to 50 hairs are extracted by a short, sharp pull with artery forceps. The anagen-to-telogen ratio is calculated. The ratio is decreased in telogen effluvium because of the increased numbers of hairs in telogen.

Scalp biopsy

This differentiates between scarring and non-scarring alopecia and also between alopecia areata and trichotillomania.

Light microscopy of hair shafts

Light and/or electron microscopy is indicated if a hair shaft defect is suspected. Skin scrapings and hair samples are taken for fungal microscopy and culture.

Hirsutism

Hirsutism is growth in the female of excess, coarse, terminal pigmented hair in androgen-dependent sites, namely in a male sexual pattern (e.g. upper lip, beard area and back; see FIG. 126.7).

Most cases are due to idiopathic hirsutism, which may follow ethnic or familial factors, or to polycystic ovarian syndrome. Other causes include adrenal hyperplasia, virilising adrenal tumours, Cushing syndrome, virilising ovarian tumours and certain medications.

The diagnostic strategy model is presented in TABLE 126.4 and followed by the key features.

Key history

History of age and rate of onset, extent and activity of the hair. Menstrual history, family history and past medical history including endocrine disorders and drugs, especially those listed above.

Key examination

• General inspection including distribution and character of the hair growth, endocrine abnormalities (e.g. Cushing syndrome), skin, abdomen and breasts

• Note other features of virilisation, specifically androgenic alopecia, acne, deepening of the voice and clitoromegaly (if indicated)

Key investigations

• Consider pituitary hormones (e.g. FSH, LH, ACTH, TSH, prolactin)

• Androgens (e.g. serum testosterone, SHBG, DHEAS, 17-OH progesterone)

• Pelvic ultrasound (polycystic ovaries)

• Urinary porphyrins

• Imaging of pituitary and adrenal regions

Routine investigation is not required unless hirsutism is severe, is of sudden onset or there are other features of virilisation.

Hirsutism of reasonably sudden onset is an important marker for serious underlying pathology. It is important to exclude adrenal or ovarian pathology.

For patients with severe hirsutism and regular menses, serum testosterone, DHEAS and early morning 17-hydroxyprogesterone levels should be measured. If menses are irregular, measure follicle stimulating hormone, luteinising hormone and serum prolactin.

Appropriate investigations include pelvic ultrasound to diagnose polycystic ovaries and serum testosterone and dehydroepiandrostenedione (DHEA), which indicates an adrenal cause of androgen excess if elevated.

Principles of management

• Exclude underlying adrenal or ovarian pathology.

• Support the patient to achieve an acceptable body image through appropriate advice and referral.

• Recommend appropriate cosmetic measures, which include bleaching, waxing, depilatory creams or shaving.

• Do not pluck hairs, especially around the lips and chin—plucking stimulates hair growth but shaving appears to have no effect.

• Laser may also help but seems to be most suitable for dark hair on a light skin and provides temporary removal, lasting up to 9 months.

Pharmaceutical options⁹

These include either suppression of ovarian and/or adrenal androgen production (oral contraception or dexamethasone) or inhibition of androgen action at the hair follicles (spironolactone and cyproterone acetate).

If the woman is of reproductive age and has no immediate wish for fertility, use:

• combined oral contraceptive pill (COCP): ethinyloestradiol + cyproterone or drosperinone

Cyproterone and drosperinone have anti-androgenic properties. Other COCPs may also be useful because they increase SHBG production and reduce ovarian androgen production.

Anti-androgenic drugs:¹

• spironolactone 100–200 mg (o) daily (takes 6–12 months to respond)

For women who are premenopausal or perimenopausal, use:

• cyproterone acetate 10–100 mg (o) daily for 10 days of each menstrual cycle plus an COCP

Cyproterone used alone usually leads to hypooestrogenism. Therefore, it should be combined with oestrogen unless this is contraindicated. Alternative oestrogen combination regimens are required for postmenopausal women.

Topical eflornithine HCl 11.5% (Vaniqa) cream for topical use on facial hair and hair under the chin is a newer preparation that slows hair regrowth. Apply thinly twice a day.

Hypertrichosis

Hypertrichosis is the increased growth of fine vellus or downy hair over the body that may either be generalised (usually) or localised. It is non-androgen dependent hair and does not respond to anti-androgen therapy. The cause is generally unknown—it may be primary or constitutional where it is usually apparent prior to puberty and where it is evenly distributed over the back and limbs. Prepubertal hypertrichosis is commonly familial but a positive family history is not always determined.

An important cause of secondary hypertrichosis is drugs. Drugs that are implicated include phenytoin, minoxidil, cyclosporin, interferon alpha and corticosteroids, both systemic and topical.

It is also associated with an underlying neoplasia, such as stomach cancer, when it is termed hypertrichosis lanuginosa acquisita or ‘malignant down’.

Other causes include anorexia nervosa, the menopause, systemic illness, starvation and a few rare syndromes (e.g. Cornelia de Lange syndrome). Treat the underlying cause, otherwise treatment is only necessary if it concerns the patient. Physical hair removal techniques are appropriate for hair that is non-androgen dependent.

Dandruff

Dandruff (pityriasis capitis) is mainly a physiological process, the result of normal desquamation of scale from the scalp. It is most prevalent in adolescence and worse around the age of 20.

If it is persistent with heavy scaling, seborrhoeic dermatitis and scalp psoriasis, which is distinguished by palpable plaques, are likely causes.

Dandruff and seborrhoeic dermatitis are related conditions on a continuum of severity. They are caused mainly by the fungal genus Malassezia, such as M. globosa, which produces lipases that break down sebum. They both have a common pathogenesis and are chronic recurring conditions presenting with scaling and a varying degree of itch. More importantly, they all respond to similar treatments.

Treatment

Shampoos:

• zinc pyrithione (e.g. Dan-Gard, Head and Shoulders)

  or

• selenium sulphide (e.g. Selsun)

Method: massage into scalp, leave for 5 minutes, rinse thoroughly—twice weekly.

Treatment of persistent or severe dandruff:

• coal tar plus salicylic acid compound (Sebitar) shampoo

  or

• T Gel/Ionil T plus shampoo

Method: as above, followed by Sebi Rinse or ketoconazole (Nizoral) shampoo. If persistent, especially itching, and Nizoral shampoo is ineffective, use a corticosteroid (e.g. betamethasone scalp lotion).

Dry hair

Advice to patients

• Don’t shampoo every day.

• Use a mild shampoo (labelled for ‘dry or damaged hair’).

• Use a conditioner.

• Have hair cut regularly to remove split or frayed ends.

• Avoid heat (e.g. electric curlers, hair dryers).

• Wear head protection in hot winds.

• Wear a rubber cap when swimming.

Oily hair

Advice to patients

• Shampoo daily with a ‘shampoo for oily hair’.

• Massage the scalp during the shampoo process.

• Leave the shampoo on for at least 5 minutes.

• Avoid hair conditioners.

• Avoid overbrushing.

• Attend to lifestyle factors: relaxation and balanced diet are important.

Hand-out sheets from Murtagh’s Patient Education 7th edition:

• Dandruff

• Hair loss in women

• Hirsutism

• Male pattern baldness