Graham-Little-Piccardi-Lasseur Syndrome 

  • Author: Scott Richard Albert Walsh, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 17, 2012
 

Background

In 1914, Piccardi described the first case of progressive scalp cicatricial alopecia, noncicatricial alopecia in the axilla and groin, and follicular lichen planus on the trunk and extremities, to which he gave the name cheratosi spinulosa (keratotic spinulosa). In 1915, Graham-Little published a similar case of a 55-year-old woman, referred by Lassueur of Lausanne, Switzerland.[1] Later, Feldman also reported another similar case, which he termed lichen planus et acuminatus atrophicans in 1936. Subsequently, several other cases were reported.

Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is a rare lichenoid dermatosis defined by the triad of multifocal cicatricial alopecia of the scalp; noncicatricial alopecia of the axilla and groin; and a follicular lichen planus eruption on the body, scalp, or both.

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Pathophysiology

Based on clinical and histological studies, Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is considered a variant of lichen planus consisting of follicular lichen planus (of the body and/or scalp) and lichen planopilaris (of the scalp).[2] Estimates show that at least 50% of patients with GLPLS experience at least one episode of typical oral and/or cutaneous lichen planus. Similar to lichen planus, GLPLS is likely the result of a T-cell–mediated immune response of unknown etiology, which involves destruction of keratinocytes expressing specific antigens.[3, 4]

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Epidemiology

Frequency

International

Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is relatively rare. A Medline search from 1951-2009 (all languages included) produced fewer than 40 cases of GLPLS in the literature.

Mortality/Morbidity

  • Progressive cicatricial alopecia of the scalp leading to permanent hair loss may elicit psychosocial distress in patients with Graham-Little-Piccardi-Lasseur syndrome (GLPLS).
  • GLPLS has not been associated with underlying systemic diseases or increased mortality rates.

Race

  • Most reported patients with Graham-Little-Piccardi-Lasseur syndrome (GLPLS) are middle-aged white women; however, no ethnic predisposition has been noted.

Sex

  • Reports show females are affected with Graham-Little-Piccardi-Lasseur syndrome (GLPLS) more frequently than males, although limited numbers preclude meaningful interpretation from the case reports.
  • Only a few case reports in the literature cite affected males, which may be secondary to fewer males demonstrating concern over the disease.

Age

  • Reported patients with Graham-Little-Piccardi-Lasseur syndrome (GLPLS) are aged 30-60 years.
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Contributor Information and Disclosures
Author

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, Canadian Dermatology Association, International Society of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Patricia T Ting, MD, MSc  Resident Physician, Division of Dermatology and Cutaneous Sciences, Department of Medicine, University of Alberta Faculty of Medicine and Dentistry, Canada

Patricia T Ting, MD, MSc is a member of the following medical societies: Alberta Medical Association, Canadian Dermatology Association, and Canadian Medical Association

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: American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Franklin Flowers, MD  Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, Affiliate Associate Professor of Pediatrics and Pathology, University of Florida College of Medicine

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

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and 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, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

References

  1. Graham-Little EG. Folliculitis decalvans et atrophicans. Br J Dermatol. 1915;27:183-5.

  2. Pai VV, Kikkeri NN, Sori T, Dinesh U. Graham-little piccardi lassueur syndrome: an unusual variant of follicular lichen planus. Int J Trichology. Jan 2011;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. Oct 15 2011;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. Oct 2010;19(3):39-42. [Medline].

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

  6. Ghislain PD, Van Eeckhout P, Ghislain E. Lassueur-Graham Little-Piccardi syndrome: a 20-year follow-up. Dermatology. 2003;206(4):391-2. [Medline].

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

  8. 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. Jan 12 2007;4:1. [Medline].

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

  10. 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. Jul 2004;18(4):463-6. [Medline].

  11. 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. Nov 2005;32(10):675-9. [Medline].

  12. 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. Jul 2004;151(1):84-90. [Medline].

  13. 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. Aug 1982;7(2):203-7. [Medline].

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

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

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

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

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

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

  20. Amato L, Massi D, Berti S, Moretti S, Fabbri P. A multiparametric approach is essential to define different clinicopathological entities within pseudopelade of Brocq. Br J Dermatol. Mar 2002;146(3):532-3. [Medline].

  21. Bianchi L, Paro Vidolin A, Piemonte P, Carboni I, Chimenti S. Graham Little-Piccardi-Lassueur syndrome: effective treatment with cyclosporin A. Clin Exp Dermatol. Sep 2001;26(6):518-20. [Medline].

  22. Chieregato C, Zini A, Barba A, Magnanini M, Rosina P. Lichen planopilaris: report of 30 cases and review of the literature. Int J Dermatol. May 2003;42(5):342-5. [Medline].

  23. Mirmirani P, Willey A, Headington JT, Stenn K, McCalmont TH, Price VH. Primary cicatricial alopecia: histopathologic findings do not distinguish clinical variants. J Am Acad Dermatol. Apr 2005;52(4):637-43. [Medline].

  24. Poblet E, Jimenez F, Pascual A, Pique E. Frontal fibrosing alopecia versus lichen planopilaris: a clinicopathological study. Int J Dermatol. Apr 2006;45(4):375-80. [Medline].

  25. Somani N, Bergfeld WF. Cicatricial alopecia: classification and histopathology. Dermatol Ther. Jul-Aug 2008;21(4):221-37. [Medline].

  26. Waldorf DS. Lichen planopilaris. Histopathologic study of disease. Progression to scarring alopedia. Arch Dermatol. Jun 1966;93(6):684-91. [Medline].

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