Graham-Little-Piccardi-Lasseur Syndrome

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

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.

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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]

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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, [5] 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.

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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.

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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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