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.
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]
Etiology
The etiology of Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is unknown; however, several hypotheses have been proposed, including the following:
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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. [5]
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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.
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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. [7]
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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). [8] 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. In 2016, Donovan reported a second case of GLPLS in a 40-year-old woman with complete androgen insensitivity syndrome. [9]
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Others: Neuropsychological stress, vitamin deficiency (specifically vitamin A), and altered hormone levels have been suggested because most GLPLS patients are perimenopausal or postmenopausal women.
Epidemiology
Frequency
Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is relatively rare. A Medline search from 1951-2016 (all languages included) produced fewer than 50 cases of GLPLS in the literature.
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, [10] 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.
Prognosis
Progressive cicatricial alopecia of the scalp leading to permanent hair loss may elicit psychosocial distress in patients with Graham-Little-Piccardi-Lasseur syndrome (GLPLS). Cicatricial scalp alopecia has a poor prognosis. This type of hair loss is permanent. Noncicatricial alopecia of the axilla and groin often spontaneously resolves.
Follicular lichen planus eruption on the body usually responds well to treatment; however, recurrence is not uncommon.
GLPLS has not been associated with underlying systemic diseases or increased mortality rates.
Patient Education
Educate Graham-Little-Piccardi-Lasseur syndrome (GLPLS) patients on the psychosocial aspects of progressive cicatricial alopecia. If indicated, discuss options and sources for cosmetic hairpieces to disguise end-stage scarring scalp alopecia.
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Follicular lichen planus eruption.
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Lichen planopilaris of the scalp resulting in cicatricial alopecia.
