Graham-Little-Piccardi-Lasseur Syndrome Clinical Presentation

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

History

  • Graham-Little-Piccardi-Lasseur syndrome (GLPLS) patients are usually otherwise healthy middle-aged women.
  • GLPLS is typically sporadic and nonfamilial. In 2004, Viglizzo et al reported one case of GLPLS in a 47-year-old mother and her 19-year-old daughter.[5]
  • The course of disease is slowly progressive (months to years) and often chronic. In 2003, Ghislain et al reported a 50-year-old woman who initially presented with disseminated lichen planus, which then progressed to the classic triad of GLPLS over a 20-year period.[6]
  • While the chronological course of GLPLS is variable, most patients usually present with clinical findings in the following order, called the triad of GLPLS:
    • Cicatricial alopecia of the scalp
    • Noncicatricial alopecia of the axilla and groin
    • Follicular lichen planus eruption of the body, scalp, or both
  • In most patients, cicatricial scalp alopecia does not respond to medical interventions and results in progressive and permanent patchy hair loss. In contrast, follicular lichen planus eruptions usually demonstrate a good response to medical treatments.
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Physical

Symptoms from the triad of Graham-Little-Piccardi-Lasseur syndrome (GLPLS) need not be present simultaneously.

  • Cicatricial scalp alopecia is chronic and progressive through several stages:
    • Mild perifollicular erythema (with or without pruritus)
    • Follicular hyperkeratosis (keratotic and/or spiny papules)
    • Patches of cicatricial alopecia with occasional tufts of normal hair
    • Loss of residual normal tufts and hair follicles
    • Cicatricial alopecia with permanent hair loss, clinically identical to pseudopelade of Brocq, in end-stage GLPLS
  • Noncicatrizing alopecia of axilla, groin, and occasionally eyebrows and follicular lichen planus of the skin (trunk, proximal limbs), scalp, or both usually resolve without treatment.
  • Patients have a history of typical cutaneous and/or oral lichen planus.
  • In 1999, Bardazzi et al reported one case of GLPLS associated with hepatitis B vaccination and further suggested that GLPLS may also be associated with liver disease (ie, hepatitis).[7] Follicular lichen planus eruption. Follicular lichen planus eruption. Lichen planopilaris of the scalp resulting in cicaLichen planopilaris of the scalp resulting in cicatricial alopecia.
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Causes

The etiology of Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is unknown; however, several hypotheses have been proposed.

  • Immunologic: HLA-DR is one of several HLA subtypes associated with lichen planus and GLPLS. HLA antigens are hypothesized to enhance a T-cell–mediated immune response of unknown etiology. Rodríguez-Bayona et al found autoantibodies to centromere passenger protein INCENP, a protein responsible for chromosomal segregation and mitosis regulation, in one patient with GLPLS.[8]
  • Genetic: With the exception of one 2004 report by Viglizzo et al that documented a familial pattern of GLPLS correlated with the presence of HLA-DR1 in a mother and daughter,[5] reports of GLPLS are usually sporadic, without any indication of genetic predisposition.
  • Viral (hepatitis B virus): Both GLPLS and lichen planus have been reported to be rare events following hepatitis B virus vaccination. The hepatitis B virus vaccine is hypothesized to stimulate the immune system and trigger lichen planus eruptions in a nonspecific manner. Lichen planus–like eruptions have not been reported with other vaccinations.[9]
  • Hormonal: In 2004, Vega-Gutiérrez et al reported a case of GLPLS in a 19-year-old phenotypically female (genetically XY) patient with androgen insensitivity syndrome (testicular feminization).[10] While the significance of both these findings is unknown, the authors implied that a hormonal etiology may be associated with the noncicatricial alopecia of the axilla and groin observed in persons with GLPLS.
  • Others: Neuropsychological stress, vitamin deficiency (specifically vitamin A), and altered hormone levels have been suggested because most GLPLS patients are perimenopausal or postmenopausal women.
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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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