eMedicine Specialties > Dermatology > Diseases of the Adnexa

Graham-Little-Piccardi-Lasseur Syndrome

Author: Scott Richard Albert Walsh, MD, PhD, Assistant Professor, Program Director, Department of Dermatology, University of Toronto, Sunnybrook Health Sciences Centre
Coauthor(s): Patricia T Ting, MSc, MD, Dermatology Resident, Division of Dermatology and Cutaneous Sciences, Department of Medicine, University of Alberta; 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
Contributor Information and Disclosures

Updated: Oct 13, 2009

Introduction

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). 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.

Frequency

International

Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is relatively rare. A Medline search from 1951-2009 (all languages included) produced fewer than 40 cases of GLPLS in the literature.

Mortality/Morbidity

• Progressive cicatricial alopecia of the scalp leading to permanent hair loss may elicit psychosocial distress in patients with Graham-Little-Piccardi-Lasseur syndrome (GLPLS).
• GLPLS has not been associated with underlying systemic diseases or increased mortality rates.

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, 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.

Clinical

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.²
• 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.³
• 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.

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 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).⁴

Causes

The etiology of Graham-Little-Piccardi-Lasseur syndrome (GLPLS) is unknown; however, several hypotheses have been proposed.

• 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
• 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,² 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.⁶
• 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).⁷ 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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