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Graham-Little-Piccardi-Lasseur Syndrome Clinical Presentation

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

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.[6]

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.[7]

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 the following 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).[8]

See the images below.

Follicular lichen planus eruption. Follicular lichen planus eruption.
Lichen planopilaris of the scalp resulting in cica Lichen 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, including the following:

  • 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. [9]
  • 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, [6] 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. [10]
  • 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). [11] 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

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
  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. 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].

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

  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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