Alopecia areata: diagnosis and management

Report

Alopecia areata: diagnosis and management Jerry Shapiro, MD, FRCPC, and Shabnam Madani, MD

From the Division of Dermatology, School of Medicine, University of British Columbia, Vancouver, British Columbia, Canada Correspondence Jerry Shapiro, MD, FRCPC, 835 W 10th Avenue, Vancouver, British Columbia V5Z 4E8, Canada Drug names minoxidil: Rogaine

Alopecia areata (AA) is an unpredictable, recurring, nonscarring, chronic inflammatory disorder of the hair and nails. Any hair-bearing area may be affected. The cause of AA is unknown although there is an overwhelming body of evidence implicating an autoimmune response mediated by T cells to an unknown hair-associated antigen.

Clinical features Alopecia areata can manifest itself with several different clinical features. Patients usually complain of abrupt hair loss and marked hair shedding. Frequently, patients will present to the physician with one or several bags of hair. The characteristic lesion of AA (Fig. 1) is commonly a round or oval, totally bald, smooth patch involving the scalp or any hair-bearing area on the body. The patch may have a mild peachy color. A frequent feature of AA patch are exclamation mark hairs that may be present at its margin. Exclamation mark hairs are broken, short hairs that taper proximally. The pull test may be positive at the margins of the patch indicating very active disease. Although hair loss is usually asymptomatic in most cases, some patients describe parasthesias with mild to moderate pruritus, tenderness, burning sensation, or pain before the appearance of the patches. The clinical presentation of alopecia areata is subcategorized according to the pattern or extent of the hair loss. If categorized according to pattern the following forms are seen: patchy AA, round or oval patches of hair loss (most common); reticular AA (Fig. 2), reticulated pattern of patchy hair loss; ophiasis band-like AA (Fig. 3), hair loss in parieto-temporo-occipital scalp; ophiasis inversus (Fig. 4), a rare band-like pattern © 1999 Blackwell Science Ltd

of hair loss in fronto-parieto-temporal scalp;1 and diffuse AA (Fig. 5), a diffuse decrease in hair density. If categorized according to the extent of involvement, the following forms may be seen: alopecia areata, partial loss of scalp hair; alopecia totalis, 100% loss of scalp hair; alopecia universalis (Fig. 6), 100% loss of body hair. Most patients present with the limited patchy type that is easily camouflaged. The initial regrowth in AA is often white followed by repigmentation (Fig. 7). Both regrowth in one site and extension of the alopecia on another site may be seen at the same time in the same patient. Patients are frequently quite young. Sixty per cent present with their first patch under the age of 20.2 Those individuals who present under the age of 10 have a poorer long-term prognosis.

Figure 1 A characteristic patch of AA with exclamation mark hairs (photo courtesy of Dr Harvey Lui) International Journal of Dermatology 1999, 38 (Suppl. 1), 19–24

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Figure 2 Reticular AA

Figure 4 Ophiasis inversus

Figure 3 Ophiasis band-like AA

Nail dystrophy may be associated with AA. The reported incidence of onychodystrophy in AA ranges from 10% to 66%3 depending how diligently it is looked for. Changes may be seen in one, multiple, or all of the nails. The dystrophy may precede or follow resolution of the AA. Pitting with irregular pattern or in organized transverse or longitudinal rows, trachyonychia (longitudinal striations resulting in sandpaper appearance), Beau’s lines (grooves through the nail matching that of lunula margin), onychorrhexis (superficial splitting of the nail extending to the free edge), thinning or thickening (pseudomycotic), onychomadesis (onycholysis with nail loss), koilonychia (concave dorsal nail plate), punctate or transverse leukonychia, and red spotted lunula (Fig. 8) may be associated with AA.4 AA may be associated with other autoimmune diseases such as atopy, thyroid disease, and vitiligo. The relationship is correlational and not causal. For instance, correcting the thyroid condition will not correct the AA. It is more likely that these correlations are a reflection of an autoimmune International Journal of Dermatology 1999, 38 (Suppl. 1), 19–24

Figure 5 Diffuse AA

diathesis that determined.

may

be

predominantly

genetically

Prognosis The only predictable thing about the progress of the AA is that it is unpredictable. Patients usually present with several episodes of hair loss and hair regrowth during their life time. The recovery from hair loss may be complete, partial, or nonexistent. Fifty per cent will regrow their hair entirely within 1 year without treatment; however, 7–10% will eventually end up with the severe chronic form of the © 1999 Blackwell Science Ltd

Shapiro and Madani

Alopecia areata Report

Figure 8 Red spotted lunula

Table 1 Protocol for treatment of adult AA* Figure 6 Alopecia universalis

Patients with less than 50% hair loss 1 Do nothing 2 Intralesional triamcinolone acetonide injections 3 Minoxidil 5% solution 4 Combination of minoxidil 5% solution and superpotent topical corticosteroid 5 Combination of minoxidil 5% solution and anthralin 6 Topical immunotherapy if the above do not work Patients with more than 50% hair loss 1 Topical immunotherapy with diphencyprone 2 Minoxidil 5% solution plus superpotent topical corticosteroids 3 Minoxidil 5% solution plus anthralin 4 PUVA 5 Systemic corticosteroid therapy (rarely)

* University of British Columbia Hair Research and Treatment Center, 1998. Figure 7 White regrowth in initial AA

condition. Poor prognostic indicators are atopy, the presence of other immune diseases, positive family history, young age of onset, and ophiasis.

the typical pattern of balding, and shedding is not prominent. The pull test is usually negative for AGA. A 4 mm punch biopsy may be necessary to make a definitive diagnosis in some cases, especially with diffuse AA. A peribulbar lymphocytic infiltrate (swarm of bees) with no scarring is characteristic of the diagnosis of AA (Fig. 9).

Differential diagnosis

Treatment plan

The differential diagnosis is usually between telogen effluvium or androgenetic alopecia (AGA). In telogen effluvium, hair loss is generalized over the whole scalp, whereas in AA it is usually patchy. Hairs that are shed are either telogen or dystrophic anagen in AA, and purely telogen in telogen effluvium. Patients with AGA usually demonstrate

At present, all treatments are palliative, only controlling the problem, and certainly not curing the condition. All local treatments may help the treated areas, but do not prevent further spread of the condition. All treatment plans for patients depend on two major factors: the extent of scalp involvement and the age of the patient. At the

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Figure 9 Peribulbar lymphocytic infiltrate (swarm of bees)

Shapiro and Madani

Figure 11 Initial DPCP application on one half of the scalp

with no scarring in AA

Figure 10 Intralesional corticosteroid injections into the

alopecic patches Figure 12 At week 24, cosmetically acceptable hair growth

post-DPCP treatment on left half of the scalp

University of British Columbia Hair Clinic patients are divided into those less than 10 years of age and those over 10 years of age. Patients over 10 are then subdivided into those with less than 50% scalp hair loss and those with more than 50% scalp hair loss. For those with less than 50% scalp hair loss, the following options are offered. First and foremost we offer the patient no treatment, as many of these patients will regrow their hair without treatment; however, most of our patients are well motivated and want treatment. First line therapy for scalp AA is intralesional corticosteroid injections into the alopecic patches. We use triamcinolone acetonide 5 mg/ml for a total of 2 ml and make 20 injections of 0.1 ml each with a 30-guage needle (Fig. 10). This is usually done every 4–6 weeks. Responses are usually seen at 1–2 months. If there is no response after 3–4 months, we will add minoxidil 5% solution BID and, in addition to the injections, also apply a superpotent corticosteroid cream such as clobetasol International Journal of Dermatology 1999, 38 (Suppl. 1), 19–24

propionate 30 min after the minoxidil. We call this triple therapy, namely monthly corticosteroid injections and twice daily minoxidil and corticosteroid cream. If there is no benefit, another option is short contact anthralin therapy with anthralin 1.0% cream applied for up to 1 h daily combined with topical minoxidil 5% solution applied twice daily. The efficacy of minoxidil solution may be enhanced with anthralin.5 For those patients with more than 50% scalp involvement, our first line is topical immunotherapy with diphencyprone (DPCP). The exact mechanism of action is unknown; however, it is believed to cause antigenic competition with the putative hair-associated antigen.6 This is presently being investigated in animal rodent models. DPCP is applied once weekly (Fig. 11). Regrowth is initially © 1999 Blackwell Science Ltd

Shapiro and Madani

Figure 13 Marked eczema after DPCP treatment, spreading

Alopecia areata Report

Figure 15 Lymphadenopathy after DPCP treatment

to the untreated area

Figure 14 Blister formation after DPCP treatment Figure 16 Pigmentary changes after DPCP treatment

seen at week 12 and cosmetically acceptable hair regrowth usually occurs by week 24 (Fig. 12). The success rate is 40%–60% for those with 50%–99% scalp involvement.7,8 If there is no response by 24 weeks, the topical immunotherapy is discontinued.9 Side-effects include marked dermatitis (Fig. 13), blister formation (Fig. 14), cervical adenopathy (Fig. 15), and pigmentary changes (Fig. 16). Other options that can be offered to the patient are systemic PUVA, minoxidil 5% solution, short contact anthralin, and superpotent topical steroids. Systemic corticosteroids are rarely used in our clinic due to their side-effect profile and the fact that steroids may be necessary for long periods and do not change the ultimate prognosis. A scalp prosthesis should be available to all patients with more than 50% scalp involvement. It should be emphasized to the patient that a prosthesis does not imply permanent hair loss, but © 1999 Blackwell Science Ltd

having one on hand is comforting for episodes of extensive hair loss. For children under the age of 10 treatment options include minoxidil 5% solution with or without topical midpotent corticosteroids or short contact anthralin therapy. It is important to address the psychologic impact of the condition. It is imperative that the physician spend sufficient time with the patient, just as one would with a patient who has recently been diagnosed as diabetes. The National Alopecia Areata Foundation (710 C Street, Suite 11, San Rafael, California 9490123853) offers patients and physicians information in the form of brochures, bimonthly newsletters, research updates, sources for scalp prosthesis, children penpals, videos for children to take to school, and International Journal of Dermatology 1999, 38 (Suppl. 1), 19–24

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information about support groups that are present in many large cities in the USA and Canada.

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References 1 Muralidhar S, Sharma VK, Kaur S. Ophiasis inversus – A rare pattern of alopecia areata. Pediatric Dermatol 1998; 15: 326–327. 2 Price V. Alopecia areata: clinical aspects. J Invest Dermatol 1991; 96: 68S. 3 Oremlhia G, Douville H, Marelli MA. Nail changes and alopecia areata. Ital Gen Rev Dermatol 1988; 25: 179– 184. 4 Berker DAR, Baran R, Dawber RPR, eds. Handbook of

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Diseases of the Nails, their Mangement. Oxford: Blackwell Science, 1995: 76–77. Fiedler V, Vendrow A, Szunpar G. Treatment resistant alopecia areata. Arch Dermatol 1990; 126: 756–759. Happle R. Antigenic competition as a therapeutic concept for alopecia areata. Arch Dermatol Res 1980; 267: 109–114. Shapiro J, Tan J, Ho V. Treatment of chronic severe alopecia areata with diphenylcyclopropenone and 5% minoxidil: a clinical and immunopathologic evaluation. J Am Acad Dermatol 1993; 29: 729–735. Van der Steen P, Van Baar H, Perret C. Treatment of alopecia areata with diphencyclopropenone. J Am Acad Dermatol 1991; 24: 253–257. Shapiro J, Price V. Hair regrowth: Therapeutic agents. Dermatologic Ther 1998; 16: 341–356.

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