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Graham-Little-Piccardi-Lasseur Syndrome Workup

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

Laboratory Studies

Some epidemiological studies describe a loose association between mucocutaneous lichen planus and hepatitis C. However, this association has not yet been described in case reports of Graham-Little-Piccardi-Lasseur syndrome (GLPLS).

Initial investigations may include antinuclear antibodies (ANA), antiextractable nuclear antigens (AENA), hepatitis B and C serology, and liver function tests to exclude other associated systemic causes of cicatricial alopecia.

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Imaging Studies

No imaging studies are required for Graham-Little-Piccardi-Lasseur syndrome (GLPLS).

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Procedures

Punch biopsies of the scalp parallel to hair shaft growth (or directed at 45° angle in African Americans) may confirm the presence of cicatricial alopecia. Multiple biopsy samples may be required for transverse (horizontal) and vertical sections, and immunohistochemistry. Histologically, the end-stage lesions in Graham-Little-Piccardi-Lasseur syndrome (GLPLS) are indistinguishable from those of pseudopelade of Brocq, discoid lupus erythematosus, folliculitis decalvans, frontal fibrosing alopecia, and other forms of cicatricial alopecia.

A skin biopsy of associated follicular papules may reveal the presence of histologic findings that correlate with the triad of GLPLS.

The presence of noncicatricial alopecia in the axilla and groin can usually be diagnosed clinically, and further skin biopsies are not necessary.

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Histologic Findings

In the early stage, an inflammatory perifollicular lichenoid infiltrate can be observed and is often associated with the infundibuloisthmic (bulge) region of the follicle.[12] This infundibuloisthmic region contains stem cells responsible for regenerating the lower two thirds of the hair follicle, which is nonpermanent. The bulb region is spared. In end-stage Graham-Little-Piccardi-Lasseur syndrome (GLPLS), atrophic dermis and fibrosed and empty hair shafts can be seen. One additional histological finding is keratinous follicular plugs with loss of sebaceous glands.

A lichenoid lymphocytic infiltrate composed of mostly CD8 and CD4 T cells may irreversibly damage stem cells and hair follicles. Profibrogenic cytokines such as interleukin (IL)–4 and IL-6, transforming growth factor (TGF)–beta, and interferon (IFN)–gamma have been reported in association with lichen planopilaris of the scalp.[13]

Direct immunofluorescence studies have reported nonspecific immunoglobulin M, and occasionally immunoglobulin G and immunoglobulin A, at the hair follicle infundibulum and isthmus, as well as linear fibrinogen deposition along the dermoepidermal junction.[14]

Histopathology of follicular papules

Findings include (1) lichenoid lymphocytic infiltrate in the upper dermis, (2) hyperkeratosis and focal hypergranulosis, (3) acanthosis with occasional saw-toothed rete ridges, and (4) dyskeratotic keratinocytes (Civatte or colloid bodies).

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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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