Graham-Little-Piccardi-Lasseur Syndrome

Discussion in 'Alopecia (areata, totalis, universalis, scarring)' started by Angela, May 11, 2009.

  1. Angela

    Angela Moderator

    Background

    In 1914, Piccardi described the first case of progressive scalp cicatricial alopecia, noncicatricial alopecia in the axilla and groin, and follicular lichen planus (LP) on the trunk and extremities, to which he gave the name cheratosi spinulosa (keratotic spinulosa). In 1915, Graham-Little1 published a similar case of a 55-year-old woman, referred by Lassueur of Lausanne, Switzerland. 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 LP eruption on the body, scalp, or both.
    Pathophysiology

    Based on clinical and histological studies, GLPLS is considered a variant of LP consisting of follicular LP (of the body and/or scalp) and lichen planopilaris (LPP, of the scalp). Estimates show that at least 50% of patients with GLPLS experience at least one episode of typical oral and/or cutaneous LP. Similar to LP, 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

    GLPLS is relatively rare. A Medline search from 1951-2007 (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 the patient.
    • GLPLS has not been associated with underlying systemic diseases or increased mortality rates.
    Race


    • Most reported patients are middle-aged white women; however, no ethnic predisposition has been noted.
    Sex


    • Reports show females are affected 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 are aged 30-60 years.
    Clinical

    History


    • Patients are usually otherwise healthy middle-aged women.
    • GLPLS is typically sporadic and nonfamilial. In 2004, Viglizzo et al2 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 al3 reported a 50-year-old woman who initially presented with disseminated LP, 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 LP 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 LP eruptions usually demonstrate a good response to medical treatments.
    Physical

    Symptoms from the triad of 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 LP of the skin (trunk, proximal limbs), scalp, or both usually resolve without treatment.
    • Patients have a history of typical cutaneous and/or oral LP.
    • In 1999, Bardazzi et al4 reported one case of GLPLS associated with hepatitis B vaccination and further suggested that GLPLS may also be associated with liver disease (ie, hepatitis).
    Causes

    The etiology of GLPLS is unknown; however, several hypotheses have been proposed.



    • Immunologic: HLA-DR is one of several HLA subtypes associated with LP and GLPLS. HLA antigens are hypothesized to enhance a T-cell–mediated immune response of unknown etiology.
    • Genetic: With the exception of one 2004 report by Viglizzo et al2 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 LP 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 LP eruptions in a nonspecific manner. LP-like eruptions have not been reported with other vaccinations.
    • Hormonal: In 2004, Vega-Gutiérrez et al5 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.
    http://emedicine.medscape.com/article/1073743-overview