Graham-Little-Piccardi-Lasseur Syndrome Clinical Presentation
- Author: Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada); Chief Editor: Dirk M Elston, MD more...
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.
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.
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.
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).
See the images below.
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. 
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,  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. 
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).  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.
Graham-Little EG. Folliculitis decalvans et atrophicans. Br J Dermatol. 1915. 27:183-5.
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].
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].
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].
Viglizzo G, Verrini A, Rongioletti F. Familial Lassueur-Graham-Little-Piccardi syndrome. Dermatology. 2004. 208(2):142-4. [Medline].
Ghislain PD, Van Eeckhout P, Ghislain E. Lassueur-Graham Little-Piccardi syndrome: a 20-year follow-up. Dermatology. 2003. 206(4):391-2. [Medline].
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].
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].
Rebora A, Rongioletti F, Drago F, Parodi. Lichen planus as a side effect of HBV vaccination. Dermatology. 1999. 198(1):1-2. [Medline].
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].
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].
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].
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].
Bottoni U, Innocenzi D, Carlesimo M. Treatment of Piccardi-Lassueur-Graham Little syndrome with cyclosporin A. Eur J Dermatol. 1995. 5:216-9.
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].
Boyd AS, King LE Jr. Thalidomide-induced remission of lichen planopilaris. J Am Acad Dermatol. 2002 Dec. 47(6):967-8. [Medline].
George SJ, Hsu S. Lichen planopilaris treated with thalidomide. J Am Acad Dermatol. 2001 Dec. 45(6):965-6. [Medline].
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].
Büyük AY, Kavala M. Oral metronidazole treatment of lichen planus. J Am Acad Dermatol. 2000 Aug. 43(2 Pt 1):260-2. [Medline].