The first step in the treatment of discoid lupus erythematosus is to rule out the presence of systemic lupus erythematosus on the basis of a complete medical history and the results of antinuclear antibody and kidney function tests. Discoid lupus erythematosus is treated with similar therapies whether it affects the scalp or the skin.11–13 The standard treatment is twice-daily application of topical high-potency corticosteroids, such as betamethasone dipropionate 0.05% or clobetasol propionate 0.05% in cream, gel, lotion, or foam (Table 2) and deep dermal injection every 4 to 6 weeks of 4 to 10mg/mL of triamcinolone acetonide. The solution is prepared by dissolving 0.1 to 0.2mL from a 40-mg vial of triamcinolone acetonide in 0.8 to 0.9mLof saline solution. The resulting dilution is then injected into the plaques (0.1 mL per cm2, maximum 2 mL) with an insulin syringe and needle. Treatment with potent topical corticosteroids can leave permanent areas of hypopigmentation and telangiectasia and the injections may cause atrophy. If there is no response within 8 to 12 weeks, oral antimalarial agents may be added. Antimalarials are the first-line option in the case of rapidly progressive alopecia. Treatment is usually started with hydroxychloroquine because of its lower ocular toxicity14 and patients should have a baseline eye examination. Smokers should be advised to stop smoking before starting treatment because it has been observed that tobacco diminishes response to treatment in a dose-dependent fashion.15 The initial dose of hydroxychloroquine is 200 to 400mg/d (4-6mg/kg/d in children), and improvement is usually observed within 4 to 8 weeks. A certain degree of repopulation of the hair can even be achieved if treatment is started promptly. If the outbreak is very severe, oral prednisone 1mg/kg can be added until disease is controlled, gradually tapering the dose over 8 weeks. In selected cases a combination of antimalarial drugs may be indicated because of their synergistic effect.16 Treatment should be maintained over a prolonged period until a few weeks after the inflammation has resolved, when the result of gradual withdrawal can be tested. In cases of resistant disease, treatment with oral retinoids may be useful. Even though acitretin and hydroxychloroquine have been shown to be equally effective,14 it is preferable to start with isotretinoin at a dosage of 1mg/kg/d17 because it causes less telogen effluvium. Moreover, isotretinoin has a shorter half-life, which reduces the risk of teratogenicity in women of childbearing age. In some cases, low maintenance doses are needed to control the disease over a prolonged period (10-40mg/d). Marked improvement has also been reported with thalidomide at doses between 50 and 300mg/d, although low maintenance doses of 25 to 50mg/d are usually required because relapse occurs early and frequently.18 The main adverse effects of this drug are teratogenicity and peripheral neuropathy.

Some studies have also shown improvement in the lesions of discoid lupus erythematosus with topical immune modulators, such as pimecrolimus 1% cream applied twice daily for 8 weeks19,20 and tacrolimus 0.1%. In a comparative study of tacrolimus 0.1% and clobetasol propionate 0.05%, no significant differences in effectiveness between the 2 treatments were found.21 However, the usefulness of these results is debatable owing to the small number of patients in the study.

Patients must also be advised to avoid exposure to sunlight during peak hours and to use photoprotective measures in the form of a wide-brimmed hat, clothes that cover the neck and shoulders, and regular application of sunscreen with a high solar protection factor.

Other drugs that have been used to treat discoid lupus erythematosus are dapsone, mycophenolate mofetil, azathioprine, gold, clofazimine, and tazarotene.11,22,23 Some authors have expressed concern about the use of biologic agents in lupus erythematosus because it has been observed that anti-TNF drugs such as etanercept may induce lupus.24 Biologic agents also favor the appearance of circulating antibodies and predispose patients to infection by mycobacteria.

Before starting any treatment, the first step is to rule out the possibility that the patient's condition is induced by a drug—in particular gold,27 atabrine, or quinacrine—or by vaccination for hepatitis B.28 Hepatitis C virus infection must also be ruled out.

Initial treatment is usually either potent topical corticosteroids29,30 (Table 2) or injections of triamcinolone acetonide (3-10mg/mL, maximum 2mL) every 4 to 6 weeks, or a combination of both.25 Good results are obtained in 40% of cases of mild or moderate disease.13 In a study of 30 patients, 2 out of 3 patients achieved remission of symptoms with a 12-week course of topical corticosteroids using a gradually tapered dose regimen.30 The authors of another study reported partial and temporary improvement in 70% of patients.31 Systemic therapy should be reserved for treating rapidly progressive disease that fails to respond to topical corticosteroid treatment. Prednisone can be used for short periods of time at a dose of 0.5 to 1mg/kg to control the disease, with gradual tapering over 2 to 4 months.30,31 Oral ciclosporin has been used as an alternative in cases of refractory lichen planopilaris. In a series of 13 cases, an average dose of 4 to 5mg/kg/d for 4 to 6 months resulted in complete remission in 53% of patients and partial response in 23%.32 Despite the good results obtained with oral corticosteroids and ciclosporin, relapse upon withdrawal of treatment tends to occur in 60% to 80% of cases.31,33 Retinoids can also be used; marked improvement in 2 patients with lichen planopilaris was reported following treatment with low doses of oral tretinoin over several months.34

There is anecdotal evidence of the use of other treatments. In a retrospective study, disease stabilized in 6 of 11 patients treated with tetracyclines,35 a result that has led some authors to consider oral tetracyclines to be the treatment of choice because of their superior safety profile.

Frontal Fibrosing Alopecia

Frontal fibrosing alopecia refers to progressive recession of the frontal and temporal hairline accompanied, in some cases, by thinning of the eyebrows and sideburns. This condition primarily affects postmenopausal women (Fig. 4).36 Because of its clinical features (follicular hyperkeratosis and perifollicular erythema) and histologic characteristics (perifollicular lymphocytic infiltrate and lamellar fibrosis of the upper portion of the follicle), most experts believe that this condition is a variant of lichen planopilaris.37 No effective treatment has been reported, although during the hyperkeratotic phase disease appears to improve with medium- to high-potency topical corticosteroids. The therapeutic options recommended for the treatment of lichen planopilaris38,39 can be tried with the addition of oral finasteride to the topical corticosteroid therapy in women with typical female pattern hair loss. Monotherapy with oral finisteride is another option. It should be noted that finasteride is contraindicated in women of childbearing age.

Figure 4.

A, Frontal fibrosing alopecia. Hairline receding about 3cm and thinning of the eyebrows. B, Frontal fibrosing alopecia in the active phase. Erythema and follicular hyperkeratosis.

(0.26MB).

Central Centrifugal Cicatricial Alopecia

There are probably 2 variants of central centrifugal cicatricial alopecia. The most typical form, found primarily in black women,43,44 is attributed to harsh hair-care practices, including the use of traumatic combing, heat, traction, and chemical products. The other form, more often found in white patients, can be considered a variant of lichen planus (Fig. 5). The fact that both forms share several clinical and histologic features with Brocq pseudopelade and folliculitis decalvans45 has led some authors to suggest that central centrifugal cicatricial alopecia may not be a separate entity but rather a clinical pattern shared by different scarring alopecias.

Figure 5.

Central centrifugal cicatricial alopecia. Cicatricial plaque of about 15cm in diameter in the interparietal region. Minimal activity at the edges of the plaque with follicular hyperkeratosis.

(0.18MB).

During the active phase, histology reveals lamellar fibroplasia and a perifollicular lymphocytic infiltrate affecting the upper portion of the hair follicle. In more advanced stages, a granulomatous perifollicular inflammation with foreign body giant cells is observed.46

The recommended treatment is avoidance of traumatic hair-care practices, if applicable, and, during the inflammatory phase, application of potent topical corticosteroids alone or in combination with oral tetracyclines (tetracycline hydrochloride 500mg/12h or doxycycline 100mg/12h).47 Treatment results in rapid improvement that can be maintained for several months, although most patients experience several relapses over a few years before the disease resolves completely, leaving oval scarred plaques, which can measure up to 10cm in length.

The first step in treatment should be to culture the pustules and obtain a sensitivity report. In patients with a history of recurrent infections; immunodeficiency must be ruled out. Folliculitis decalvans responds well to antistaphylococcal antibiotics (Table 3), cloxacillin, fusidic acid, clindamycin alone or in combination with rifampicin or amoxicillin clavulanate,53,54 and to broad-spectrum antibiotics and antineutrophil agents. However, the condition usually recurs when treatment is discontinued. In recent years, remissions of 2 years or more have been achieved with the introduction of combination therapies based on rifampicin53 because of their excellent bactericidal action and improved intracellular penetration.55,56 Rifampicin has been reported to eliminate nasal carriage of S aureus.57 Patients using this drug must be warned about the orange coloration they may observe in their urine and the possibility of developing a hypersensitivity syndrome with eosinsophilia.56,58,59 However, the use of rifampicin monotherapy should be avoided because this regimen facilitates the development of resistance.57 One of the most commonly used combinations is rifampicin (300mg/12h) + clindamycin (300mg/12h) for 10 weeks. Clindamycin can cause diarrhea and even pseudomembranous colitis caused by Clostridium difficile, a bacteria that is becoming increasingly aggressive.60 Rifampicin can also be used in combination with ciprofloxacin or clarithromycin. Oral fusidic acid appears to be equally effective alone or in combination with fusidic acid ointment (500mg/d for 3 weeks).61 Prolonged treatment with fusidic acid can cause gastrointestinal disorders.62 Liver function should be monitored, and this therapy should be avoided in patients with liver disease. Very good results have been obtained with broad-spectrum antibiotics, such as tetracyclines and trimethoprim-sulfamethoxazole. If these are used, a topical antibiotic, such as fusidic acid or mupirocin, should be added to eradicate nasal S aureus.63 Isotretinoin does not appear to be very effective, although 1 patient responded to a combination regimen of isotretinoin, prednisolone, and clindamycin.63 Some cases have responded to treatment with oral dapsone at doses of 75 to 100mg/d,64 but this treatment must be maintained over a prolonged period at a low dose to prevent relapse. Laser hair removal and radiotherapy at anti-inflammatory doses have also been tested with good results.65,66 Several treatment cycles may be necessary to achieve prolonged remission.

Prolonged remission has been achieved with isotretinoin, the first-line treatment.67 The recommended dose is 1mg/kg/d for 4 months, followed by 0.75mg/kg/d for a further 6 months. Some patients with intolerance to this regimen have responded equally well to continuous treatment with low doses of isotretinoin (10mg/d).70 Resistant cases have been reported to respond to combinations of oral rifampicin and isotretinoin71 and oral isotretinoin and dapsone.72 A single case of resistant disease successfully controlled with infliximab has been reported.73 Another case responded favorably to treatment with topical isotretinoin.74

Tufted folliculitis is a unusual form of progressive and recurrent folliculitis that resolves leaving irregular patches of scarring alopecia.75

The etiology and pathogenesis of this condition are poorly understood and there is debate about whether it is in fact a separate nosologic entity.76 It is thought that it might be related to an inappropriate immune response to S aureus, since this pathogen has been isolated in most of these follicular lesions. Clinically, tufted folliculitis is characterized by tufts of hair growing on patches of scarring alopecia, giving the scalp a characteristic doll's head appearance.77

Histopathologic findings include a perifollicular inflammatory infiltrate located in the upper and mid-dermis. Hair detritus is also observed in the cytoplasm of macrophages and in multinucleate giant cells.76–78 This condition has usually been treated with topical and systemic antibiotics, especially antistaphylococcal agents; however, complete cure is rare. Powell et al54 used an antibiotic regimen combining rifampicin 300mg/12 h and clindamycin 300mg/12h for 10 weeks, which was effective in 10 out of 18 patients.54 Other treatments that have been used with limited success include oral isotretinoin, corticosteroids, and zinc sulfate. Surgery may be used to manage localized lesions that do not respond to medical treatment.

Early diagnosis is important in the treatment of acne keloidalis because it allows the patient to start aggressive treatment to reduce the risk of scarring, to minimize traumas (rubbing caused by the collars of shirts and t-shirts, shaving the scalp, and very short hair cuts), and to avoid exposure to the harsh chemical hair products used in some traditional remedies.79,80 Incipient disease can be treated with potent topical corticosteroids, such as clobetasol propionate 0.05% in lotion or foam either alone82 or in combination with topical antibiotics.83 The papular and papulonodular forms can be controlled with monthly injections of triamcinolone acetonide (10mg/mL) alone or in combination with oral antibiotics (clindamycin or tetracyclines such as minocycline or doxycycline 100mg/12-24h or cefradroxil 500mg/12h after the results of culture and sensitivity testing have been received) (Table 3). Rifampicin has also been shown to be effective, although this agent should never be used in single drug therapy.83,84 The use of antiseptic soaps such as chlorhexidine is also recommended as a complement to these treatments. There is no evidence that intralesional etanercept is more effective than injection of triamcinolone.85

Surgery is the treatment of choice in patients with very extensive keloidal plaques. Some experts have recommended treating keloidal papules with diode laser hair removal techniques once the bacterial infection has been cleared. This approach has resulted in a 90% to 95% improvement after 4 treatment sessions over 1 or 2 months with no new lesions during a 6-month follow-up84; however the usefulness of this treatment regimen is still a matter of debate.83