Case Study Answers
1. What differential diagnoses are you considering for this child?
Normal hair loss from the scalp is about 100 to 125 hairs a day and does not have a noticeable effect on appearance, but an excess loss will result in baldness (Springer et al., 2003, Yang et al., 2004). The term "alopecia" is used to describe human hair loss sometimes to the point of baldness. Alopecia may involve the entire scalp (alopecia totalis) or the entire body (alopecia universalis). When evaluating hair loss, recent physical or psychological stress should be included in the detailed history relating to the hair loss (Springer et al., 2003). The clinician also should evaluate when the hair loss started and if it was gradual or falling out in clumps (Springer et al., 2003).
The patient did not have any complaints of hormone abnormalities such as depression, hirsutism, or menstrual abnormalities. Hair loss or thinning can be found in patients with estrogen dominance or high levels of estrogen, which also elicits symptoms of migraines, mood swings, weight gain, fatigue, insomnia, and irregular periods. When symptoms of hormonal abnormalities are present, prolactin, follicle-stimulating hormone, luteinizing hormone, dehydroepiandrosterone sulfate, thyroid-stimulating hormone, and other endocrine tests can be ordered to evaluate for hyperandrogenism or metabolic disorders (Springer et al., 2003, Yang et al., 2004) as a cause of the hair loss.
Tinea capitis—that is, ringworm of the scalp—must be ruled out by a close examination of the hair follicle where the hair breaks off and leads to small black dots. Tinea capitis is the most common fungal infection in children and is caused by Trichophyton tonsurans and Microsporum (Andrews & Burns, 2008). The fungus invades the hair shaft and often causes a circumscribed alopecia (Andrews & Burns, 2008). The scalp may be scaly with hairs broken at the surface and yellow crusts may be present (Watkins, 2009). Patients with tinea of the scalp often present with pruritic areas and in some instances can have pus-filled lesions or even kerions where the scalp is red and swollen. The physical examination of the scalp provided no indications that the patient would benefit from a skin scraping. If scaling or inflammation were present, a skin scraping of the area and a potassium hydroxide test could be used to detect fungal infection (Usatine, 2004).
Trichotillomania, the compulsive pulling out of one's own hair, is the most common cause of childhood alopecia (Messinger & Chang, 1999) and should be another diagnostic consideration. Trichotillomania leads to bald patches with an incomplete thinning pattern (Springer et al., 2003). These patches can be of varying size or shape, have no evidence of scarring, and broken or newly grown hairs are often seen in the area. Patients are usually between 8 and 12 years of age and often will deny that they are pulling out their own hair (Watkins, 2009). Patients sometimes eat the plucked hairs, which is known as trichophagy; this action often results in internal complications or a bowel obstruction (Springer et al., 2003).
Traction alopecia is seen when the hair is pulled too tight or is chemically damaged, which is mostly seen with the tight regular braiding of hair or tight pony-tails (Watkins, 2009). Traction alopecia is often seen in the "cornrow" grooming style, and treatment is to avoid these hairstyles. Traction alopecia also may occur in female athletes who pull their hair into tight ponytails (Springer et al., 2003).
Alopecia may also occur in patients with lupus, a chronic autoimmune disease that affects multiple body systems and may include skin and hair involvement. If the patient had other symptoms indicative for lupus erythematous, such as fever, weight change, arthralgia, malar rash, Raynaud's phenomenon, or a rash characteristic of syphilis (red-brown, rough characteristic covering of any part of the body, including the hands and feet), then serologic testing would be indicated. If the diagnosis is still unknown, a skin biopsy can be utilized.
Alopecia areata, which is an autoimmune disease, affects persons of any age and either sex, and it affects up to 2% of the population (National Alopecia Areata Foundation, 2010, Watkins, 2009). Alopecia areata may present as patches of baldness, affect the entire scalp, or even cause total loss of all body hair (Delamere, Sladden, Dobbins, & Leonardi-Bee, 2008). The scalp will appear normal on examination but upon a closer look, the hair follicle will be narrower near the base and thicker at the distal end, which is sometimes referred to as "exclamation-mark hairs," as was the case in our patient's presentation (Yang et al., 2004, Watkins, 2009). In up to 50% of cases the patient will have nail pitting with longitudinal ridging (Watkins, 2009). Other abnormal nail features may be present, such as thinning, thickening, or a red-spotted lunula (Wasserman et al., 2007).
2. What is the etiology of alopecia areata?
Alopecia areata is an autoimmune disease that involves cytokines released from lymphocytes around the hair follicle, causing the hair to be rejected (Watkins, 2009). Alopecia areata may present as patches of baldness, affect the entire scalp, or even cause total loss of all body hair (Delamere et al., 2008). An increased number of catagen and telogen follicles are present in the scalp and the hair matrix is permeated with CD4 and CD8 lymphocytes (Wasserman et al., 2007). Pernicious anemia, vitiligo, or systemic lupus erythematosus are more likely to develop (Watkins, 2009). The hair loss is highly unpredictable and can be cyclical, growing back or falling out again at any time (NAAF, 2010).
Stress is frequently cited as contributing to alopecia areata, although the exact mechanism and relationship are unknown (Bolduc et al., 2010,Paus and Arck, 2009, Watkins, 2009). Episodes of alopecia areata often occur after severely stressful life events (Paus & Arck, 2009).Paus and Arck (2009) identified the need for future studies on intracutaneous responses to stress with skin-derived neuro-endocrine feedback signals and the effect this might have on the central nervous system.
A variety of medications may cause hair loss and have been known to trigger alopecia areata. Different anti-rheumatic agents, such as auranofin, leflunomide, methotrexate, and sulfasalazine, can cause hair loss. Other medications that cause hair loss include lithium, valproate, interferons, and retinoids, including acitretin, etretinate, and isotretinoin. Although less commonly reported, other medications that may cause alopecia are angiotensin-converting enzyme inhibitors, antiarrhythmic agents, anticoagulant drugs, fluconazole, liposomal amphotericin products, antipsychotic agents, antiretroviral drugs, β-blockers, H2 blockers, statins, and antidepressant drugs.
The NAAF (2010) states that one of five persons with alopecia areata have a family member who also has experienced hair loss. Alopecia areata often may occur with a family history or in conjunction with a medical history of asthma, hay fever, eczema, or other autoimmune diseases.Yang et al. (2004) suggest a polygenic additive mode of inheritance; their research study results showed that 8.4% of the patients had a positive family history of alopecia areata.
3. What is the appropriate treatment for alopecia areata?
Hair usually grows back within 1 year in 95% of all cases, and no treatment is usually indicated or necessary (Springer et al., 2003). The hairs often initially lack pigmentation during hair regrowth and are lighter or white in appearance (Wasserman et al., 2007). Hair loss can cause psychosocial and emotional distress in patients but is otherwise a self-limiting condition.
Intralesional steroids are sometimes used to speed the re-growth of the hair but do not prevent other areas of future hair loss (Watkins, 2009). Intralesional injections are becoming the most common treatment for alopecia and often are given by dermatology specialists (National Alopecia Areata Foundation, 2010, Springer et al., 2003). The steroid is given monthly through multiple injections into the skin and around the bare areas. Triamcinolone acetonide (Kenalog) and triamcinolone hexacetonide (Aristospan) have had significant positive response rates (Wasserman et al., 2007) when given in multiple 0.1-mL shots approximately 1 cm apart into the deeper dermis, with injections given no more than 4 to 6 weeks apart. By abiding with the recommended volume per site and frequency, atrophy, the most common adverse effect of intralesional injections, can be minimized (Springer et al., 2003, Wasserman et al., 2007). Injections usually are not given for more then 6 months at a time (Springer et al., 2003).
Different topical corticosteroids often are preferred in children because they are painless and have a wide safety margin (Wasserman et al., 2007). Topical agents used in persons with alopecia areata include fluocinolone acetonide cream and scalp gel, betamethasone valerate lotion, and desoximetasone cream. A Cochrane Database review of randomized controlled trails byDelamere and colleagues (2008) found that although topical steroids and minoxidil are commonly prescribed for alopecia areata, no evidence exists that they have long-term benefits.
Chronic severe alopecia areata can be treated with topical immunotherapy. Immunomodulators include anthralin (Anthra-Derm), PUVA (psoralen plus ultraviolet light A), and the biologic response modifier minoxidil (Rogaine) (Springer et al., 2003). Anthralin can cause renal impairment and no efficacy has been established for its use in children. Psoralen or PUVA, also known as photochemotherapy, can increase the risk of squamous cell carcinomas (photocarcinogenic) and cataracts and cause skin burning and aging. Minoxidil carries a U.S. Federal Drug Administration black box warning for pericardial effusion progressing to tamponade, pericarditis, angina, and sodium and water retention (Lexi-Comp, 2010). Only clinicians with experience in prescribing these agents should use them because of their potentially severe adverse effects (Springer et al., 2003).
Minoxidil affects hair follicles by stimulating proliferation at the base of the hair. It was first used as an antihypertensive medication but caused hypertrichosis, or abnormal hair growth, which led to its treatment for forms of alopecia (Wasserman et al., 2007). A topical minoxidil solution is sometimes used in twice-a-day applications to re-grow the hair. This solution can be used on the scalp, eyebrows, and beard area. Occasionally a cortisone cream is applied 30 minutes after the application of minoxidil to both reduce inflammation and stimulate hair growth.
Anthralin cream is a tarlike substance that is often used for psoriasis and has shown improvement in treatment of some cases of alopecia areata. The tar is applied to the area of hair loss once daily and washed away 30 to 60 minutes later. Adverse effects include skin irritation and brownish discoloration of the treated skin (Thiedke, 2003,Wasserman et al., 2007).
Hair loss can cause considerable distress in both children and adults. Even small patches of alopecia may lead to anxiety and fear and need to be considered when discussing treatment with patients (Watkins, 2009). Adolescent patients are especially concerned with their appearance, which can directly relate to their self-esteem. The clinician should consider the psychosocial and psychological impact of hair loss and treat or refer as indicated.
4. What patient education would you provide for the family?
The clinician should review the possible causes of alopecia areata, including different medications, stressful life events, or a contributory family history, as well as the possibility of an autoimmune reaction. Different treatment options should be discussed and referral to a dermatological specialist should be provided, particularly someone with experience treating alopecia.
The NAAF is the leading research institute regarding alopecia areata in the United States, Canada, and Europe, and currently has provided more than $2 million in funding to numerous institutes worldwide. NAAF cosponsors an International Research Workshop on Alopecia Areata at the National Institutes of Health every 4 years to provide a forum for investigators on alopecia areata research. NAAF also provides educational information, resources, and support sites for people affected by alopecia areata (NAAF, 2010). For more information about the Foundation, visit its Web site at https://www.naaf.org (NAAF, 2010).
J Pediatr Health Care. 2011;25(4):261-265. © 2011 Mosby, Inc.