Dermatology Online Journal

Treatment of lichen planopilaris with mycophenolate mofetil
Umit Tursen MD, Hale Api MD, Tamer Kaya MD, Guliz Ikizoglu MD
Dermatology Online Journal 10 (1): 24

From the Department Of Dermatology, Faculty Of Medicine, Mersin University, Turkey. utursen@mersin.edu.tr

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Abstract

Mycophenolate mofetil (MMF) is an immunosuppressive drug that has recently been used to treat autoimmune and inflammatory skin diseases. We report the first case of lichen planopilaris (LPP) successfully treated with MMF. The treatment of our patient demonstrates a novel therapeutic option for patients with LPP; MMF treatment may be preferable to azathioprine treatment because MMF has a safer adverse-effect profile. Larger studies must be performed to establish the risk-benefit ratio of various therapeutic dosages of MMF for these patients.

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Clinical summary

Mycophenolate mofetil (MMF) is an immunosuppressive drug that has recently been used to treat autoimmune and inflammatory skin diseases [1, 2]. We report the first case of lichen planopilaris (LPP) successfully treated with MMF.

Presentation.—A 44-year-old male patient presented with an 8-month history of erythematous-to-violaceous lichenoid papules and plaques exhibiting a reticulated pattern on the fronto-parietal region of the scalp. Patch-shaped, localized, and scarring alopecia was observed on the same region. The lesions were sharply demarcated and moderately pruritic (Fig. 1). No mucosal lesions were present.

Laboratory.—The result of routine complete blood cell count, urinalysis, erythrocyte sedimentation rate, liver and kidney function tests were within normal limits. Antinuclear and anti-DNA antibodies were negative, and total C3 and C4 complement levels and immunoglobulins including IgE were normal. Hepatitis B surface antigen, anti-Hepatitis B surface antigen, anti-Hepatitis B core IgM, and anti-Hepatitis C virus antibodies were negative.

Pathology.—A skin biopsy specimen obtained from the scalp of our patient revealed compact orthokeratosis, wedge-shaped hypergranulosis of follicular infundibulum, and folliculitis. Histopathological findings of the scalp biopsy were in accordance with LPP.

Treatment.—Based on clinicopathological and laboratory findings, a diagnosis of LPP was made. Treatment with MMF (500 mg twice daily) was started. After 4 weeks the previously existing lesions decreased in thickness and no new plaques developed. By week 8 the lesions were markedly improved, but the patient still complained of significant pruritus (Fig. 2). MMF treatment was then reduced to 250 mg twice daily, and triamcinolone acetonide (1mg/kg per month) was started. Our patient experienced only arthralgia as an adverse effect; the results of routine laboratory tests were within normal limits, and his lesions underwent complete remission except for cicatricial alopecia. Therapy was discontinued after 6 months. No recurrence has been observed in a follow-up period of 3 months.

Figure 1	Figure 2
Violaceous lichenoid papules with reticular pattern and scarring alopecia located on the scalp before treatment (Fig. 1)
Clinical healing at the third month of treatment (Fig. 2)

Discussion:

Lichen planopilaris designates lichen planus with a follicular arrangement of lesions. It predominantly affects the scalp. Initially, there may be only follicular papules or perifollicular erythema; however, with progression, irregularly-shaped atrophic patches of scarring alopecia develop on the scalp. The association of scarring alopecia of hair-bearing areas and hyperkeratotic follicular papules on glabrous skin is known as Graham Little syndrome. LPP is an inflammatory mucocutaneous disease primarily mediated by T-lymphocytes [3, 4]. Current treatment options include psoralen-UV-A, systemic and topical steroids, systemic retinoids, antimalarials, and immunosuppressive agents, all of which are reported to be effective in anecdotal reports [5].

MMF is the morpholinoethylester prodrug of mycophenolic acid, an agent that inhibits the proliferation of B- and T-lymphocytes through noncompetetive, reversible inhibition of inosine monophosphate dehydrogenase, a key enzyme in the de-novo synthetic pathway of guanine nucleotides [1, 2]. MMF monotherapy or combination with the other systemic drugs may be effective therapy for patients with pemphigus vulgaris, bullous pemphigoid, pemphigus foliaceus, atopic dermatitis, necrobiosis lipoidica diabeticorum, pyoderma gangrenosum, scleroderma, systemic lupus erythematosus, and psoriasis vulgaris [6, 7, 8, 9, 10, 11, 12, 13, 14, 15].

Several clinical variants of LP exist, including hypertrophic LP, ulcerative LP, lichen planus actinicus, lichen planus pemphigoides, and LPP. MMF has been successfully used for the treatment of some clinical variants of lichen planus including ulcerative LP and lichen planus pemphigoides [16, 17].

To our knowledge, our patient is the first case with LPP case successfully treated with MMF. The treatment of our patient demonstrates a novel therapeutic option for patients with LPP; MMF treatment may be preferable to azathioprine treatment because MMF has a safer adverse-effect profile. However, larger studies must be performed to establish the risk-benefit ratio of various therapeutic dosages of MMF for these patients.

References

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