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Graham-Little-Piccardi-Lasseur Syndrome Treatment & Management

  • Author: Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada); Chief Editor: Dirk M Elston, MD  more...
 
Updated: May 10, 2016
 

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,[15, 16] thalidomide,[17, 18, 19] 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.[20]

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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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Contributor Information and Disclosures
Author

Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada) Clinical Assistant Professor, University of Calgary Faculty of Medicine, Canada

Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada) is a member of the following medical societies: Alberta Medical Association, American Academy of Dermatology, American Society for Dermatologic Surgery, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Galderma; Janssen Inc.<br/>Received income in an amount equal to or greater than $250 from: Galderma; Janssen Inc.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Franklin Flowers, MD Department of Dermatology, Professor Emeritus Affiliate Associate Professor of Pathology, University of Florida College of Medicine

Franklin Flowers, MD is a member of the following medical societies: American College of Mohs Surgery

Disclosure: Nothing to disclose.

Neil Shear, MD Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association, American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics

Disclosure: Nothing to disclose.

Scott Richard Albert Walsh, MD, PhD Assistant Professor, Program Director, Department of Dermatology, University of Toronto, Sunnybrook Health Sciences Centre

Scott Richard Albert Walsh, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, International Society of Dermatology, Society for Investigative Dermatology, Canadian Dermatology Association

Disclosure: Nothing to disclose.

References
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  2. Pai VV, Kikkeri NN, Sori T, Dinesh U. Graham-little piccardi lassueur syndrome: an unusual variant of follicular lichen planus. Int J Trichology. 2011 Jan. 3(1):28-30. [Medline]. [Full Text].

  3. Vashi N, Newlove T, Chu J, Patel R, Stein J. Graham-Little-Piccardi-Lassueur syndrome. Dermatol Online J. 2011 Oct 15. 17(10):30. [Medline].

  4. Zegarska B, Kallas D, Schwartz RA, Czajkowski R, Uchanska G, Placek W. Graham-Little syndrome. Acta Dermatovenerol Alp Panonica Adriat. 2010 Oct. 19(3):39-42. [Medline].

  5. László FG. Graham-Little-Piccardi-Lasseur syndrome: case report and review of the syndrome in men. Int J Dermatol. 2014 Aug. 53(8):1019-22. [Medline].

  6. Viglizzo G, Verrini A, Rongioletti F. Familial Lassueur-Graham-Little-Piccardi syndrome. Dermatology. 2004. 208(2):142-4. [Medline].

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  8. Bardazzi F, Landi C, Orlandi C, Neri I, Varotti C. Graham Little-Piccardi-Lasseur syndrome following HBV vaccination. Acta Derm Venereol. 1999 Jan. 79(1):93. [Medline].

  9. Rodriguez-Bayona B, Ruchaud S, Rodriguez C, et al. Autoantibodies against the chromosomal passenger protein INCENP found in a patient with Graham Little-Piccardi-Lassueur syndrome. J Autoimmune Dis. 2007 Jan 12. 4:1. [Medline].

  10. Rebora A, Rongioletti F, Drago F, Parodi. Lichen planus as a side effect of HBV vaccination. Dermatology. 1999. 198(1):1-2. [Medline].

  11. Vega Gutierrez J, Miranda-Romero A, Perez Milan F, Martinez Garcia G. Graham Little-Piccardi-Lassueur syndrome associated with androgen insensitivity syndrome (testicular feminization). J Eur Acad Dermatol Venereol. 2004 Jul. 18(4):463-6. [Medline].

  12. Mobini N, Tam S, Kamino H. Possible role of the bulge region in the pathogenesis of inflammatory scarring alopecia: lichen planopilaris as the prototype. J Cutan Pathol. 2005 Nov. 32(10):675-9. [Medline].

  13. Moretti S, Amato L, Massi D, Bianchi B, Gallerani I, Fabbri P. Evaluation of inflammatory infiltrate and fibrogenic cytokines in pseudopelade of Brocq suggests the involvement of T-helper 2 and 3 cytokines. Br J Dermatol. 2004 Jul. 151(1):84-90. [Medline].

  14. Horn RT Jr, Goette DK, Odom RB, Olson EG, Guill MA. Immunofluorescent findings and clinical overlap in two cases of follicular lichen planus. J Am Acad Dermatol. 1982 Aug. 7(2):203-7. [Medline].

  15. Bottoni U, Innocenzi D, Carlesimo M. Treatment of Piccardi-Lassueur-Graham Little syndrome with cyclosporin A. Eur J Dermatol. 1995. 5:216-9.

  16. Mirmirani P, Willey A, Price VH. Short course of oral cyclosporine in lichen planopilaris. J Am Acad Dermatol. 2003 Oct. 49(4):667-71. [Medline].

  17. Boyd AS, King LE Jr. Thalidomide-induced remission of lichen planopilaris. J Am Acad Dermatol. 2002 Dec. 47(6):967-8. [Medline].

  18. George SJ, Hsu S. Lichen planopilaris treated with thalidomide. J Am Acad Dermatol. 2001 Dec. 45(6):965-6. [Medline].

  19. Jouanique C, Reygagne P, Bachelez H, Dubertret L. Thalidomide is ineffective in the treatment of lichen planopilaris. J Am Acad Dermatol. 2004 Sep. 51(3):480-1. [Medline].

  20. Büyük AY, Kavala M. Oral metronidazole treatment of lichen planus. J Am Acad Dermatol. 2000 Aug. 43(2 Pt 1):260-2. [Medline].

 
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Follicular lichen planus eruption.
Lichen planopilaris of the scalp resulting in cicatricial alopecia.
 
 
 
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