Graham-Little-Piccardi-Lasseur Syndrome Treatment & Management

Updated: May 14, 2018
  • Author: Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada); Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Topical, intralesional, and systemic corticosteroids; retinoids; psoralen plus ultraviolet light A (PUVA); antimalarials; and tetracycline antibiotics have been used with limited success in patients with Graham-Little-Piccardi-Lasseur syndrome (GLPLS). In recent years, various case reports have documented successful treatment of GLPLS with cyclosporine A, [16, 17] thalidomide, [18, 19, 20] and metronidazole (authors' observation). No definite treatments have been developed for GLPLS. Patients tend to be treated empirically for this condition.

Corticosteroids (topical, intralesional, systemic) have not been shown to be effective in the treatment of cicatricial alopecia associated with GLPLS, although they are moderately effective for follicular lichen planus. High- and super high-potency topical corticosteroids are still the treatment of choice for mild cicatricial alopecia or in patients in whom systemic medications are contraindicated. Intralesional triamcinolone acetonide is often ineffective for the treatment of cicatricial scalp alopecia. The same results have been documented for treatment with systemic corticosteroids, for which many adverse effects are known (eg, immunosuppression, osteoporosis, avascular necrosis, Cushing syndrome, diabetes, cataracts).

Cyclosporin A was effective for the treatment of 5 eligible patients with GLPLS. The dose ranges from 3-5 mg/kg/d for 3-5 months. This dose has been reported to be more effective in the first stage of cicatricial alopecia prior to dermal alteration. A significant decrease in follicular papules, attenuation of hair loss, and patchy hair regrowth have been documented. Patients remained free of symptoms for up to 20 months following treatment, with no adverse effects reported. This medication may also be used for alopecia areata, lupus erythematosus, lichen planus, and lichen planopilaris. In 3 patients with lichen planopilaris resistant to treatment with oral hydroxychloroquine or topical and intralesional corticosteroids, cyclosporin A aborted further progression of cicatricial alopecia.

Thalidomide at 50-150 mg/d for up to 2 months has been associated with significant hair regrowth of cicatricial scalp alopecia in 2 case reports. More recently, however, thalidomide titrated up to 200 mg/d (from an initial 1-mo dose of 100 mg/d) was reported to be ineffective in a case series of 6 patients (4 with lichen planopilaris and 2 with pseudopelade of Brocq) during a 6-month open-label trial. Previously, thalidomide has been successful in attenuating and reversing immune-mediated alopecia associated with discoid lupus erythematosus and erosive mucosal lichen planus.

Oral metronidazole at 500 mg twice daily for 8 weeks was used successfully at the authors' center for a female patient with GLPLS, with notable resolution of follicular papules and attenuation of cicatricial alopecia. To the authors' knowledge, this is the first report of GLPLS treated with metronidazole. In a 2000 study by Büyük and Kavala of patients with generalized lichen planus, 13 (68%) of 19 patients showed complete response to 500 mg of oral metronidazole administered twice daily. [21]

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Long-Term Monitoring

Regular follow-up is recommended to assess the course of Graham-Little-Piccardi-Lasseur syndrome (GLPLS) and the effectiveness of prescribed treatments. Ask patients about any adverse effects to medications and manage accordingly.

Conduct appropriate laboratory studies as indicated with the use of systemic medications (ie, cyclosporine, thalidomide). Take necessary steps to prevent permanent cicatricial alopecia.

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