Case Letter

Graham-Little-Piccardi-Lassueur Syndrome

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Practice Points

  • Graham-Little-Piccardi-Lassueur syndrome (GLPL) is characterized by a triad of cicatricial alopecia of the scalp, nonscarring alopecia of the axillae and/or groin, and a rough follicular eruption on the body and/or scalp.
  • Graham-Little-Piccardi-Lassueur syndrome is considered one of the 3 clinical variants of lichen planopilaris.
  • Potential therapies for GLPL include hydroxychloroquine, cyclosporine, tetracyclines, and pioglitazone.


 

References

To the Editor:

A 56-year-old white woman with a history of melanoma and hypertension presented for evaluation of progressive hair loss of more than 1 year’s duration with associated pruritis. Scalp examination revealed diffuse erythema and scarring alopecia of the bilateral parietal and temporal regions. Physical examination also revealed nonscarring alopecia of the bilateral axillae, with associated thinning of the pubic hair, eyebrows, and eyelashes, as well as keratosis pilaris on the upper arms. Biopsy of the parietal scalp revealed mild scarring alopecia with isthmic fibroplasia consistent with early lichen planopilaris (LPP)(Figure). These histologic features combined with the patient’s clinical presentation were consistent with a diagnosis of Graham-Little-Piccardi-Lassueur syndrome (GLPL).

Graham-Little-Piccardi-Lassueur syndrome was first described by Piccardi in 1913.A second case was then described by Graham-Little in 1915 in a patient referred by Lassueur, resulting in the name it bears today.1,2 The condition presents most commonly in middle-aged white women and is characterized by a triad of cicatricial alopecia of the scalp, nonscarring alopecia of the axillae and/or groin, and a rough follicular eruption on the body and/or scalp. Symptoms may not be present simultaneously. In GLPL, scarring alopecia of the scalp often precedes follicular eruptions of the trunk, arms, and legs by as much as years,2 and the inverse also has been reported.1 The inflammatory lesions of the scalp eventually resolve spontaneously, but the hair loss is by definition irreversible.

This rare condition is considered one of the 3 clinical variants of LPP. Other variants include classic LPP, also known as follicular lichen planus, and frontal fibrosing alopecia.3 More recently, fibrosing alopecia in a pattern distribution has gained some popularity as a fourth variant of LPP.4 All variants of LPP, including GLPL, result in a scarring alopecia. The classic scalp finding is an erythematous to violaceous, perifollicular, hyperkeratotic scale at the base of the terminal hairs. The population of inflamed follicles spreads outward, leaving behind a round to oval, central, atrophic scar that often is devoid of follicles. Few hairs may persist within zones of alopecia at presentation; however, these hairs are affected by inflammation and also will likely shed. A hair pull test will be positive at the margins during active disease, consisting of mostly anagen hairs on trichogram examination.1,5 Patients may develop only a single foci of hair loss, but much more commonly, a patchy multifocal alopecia is noted.6 Sites often will coalesce. Onset of scalp alopecia may be insidious or fulminant.

The nonscarring alopecia of the axillae and groin may be described as subtle thinning to complete hair loss with no signs of atrophy or inflammation. Although not commonly reported, a case of nonscarring alopecia located on the shoulders has been seen.7

The follicular eruption that can be present on the trunk, arms, or legs in GLPL is most often but not limited to keratosis pilaris, as was seen in our patient. One reported case also described lichen spinulosus as a potential variant.8 Lichen planopilaris is separate from lichen planus (LP) because of its selective follicular involvement vs the nonselective mucocutaneous distribution of LP. The 2 processes also are histologically distinct; however, estimations have shown that more than 50% of patients with GLPL experience at least 1 episode of mucosal or cutaneous LP in their lifetime.9 Rarely, coexistence of GLPL and LP lesions has been described. One reported case of GLPL and concomitant hypertrophic LP could represent a severe form of the disease.9 Additionally, lichen planus pigmentosus, an uncommon variant of LP characterized by hyperpigmented brown macules in sun-exposed areas and flexural folds, was identified in a case report of an Asian woman with GLPL.10

As a general rule, the variants of LPP most commonly are seen in postmenopausal women aged 40 to 60 years; however, rare cases in a child and a teenager have been reported.11 The GLPL variant of LPP is reported up to 4 times more frequently in females.5 Pruritus and pain are inconsistent findings, and there are no systemic signs of illness. A case of androgen insensitivity syndrome associated with GLPL suggested a potential influence of hormones in LPP.12 Stress, vitamin A deficiency, and autoimmunity also have been proposed as triggers of GLPL.13 Furthermore, familial GLPL was described in a mother and daughter, though the association was uncertain.14 Our patient had no relevant family history.

Workups to reveal the etiology of GLPL have been inconclusive. Reports of laboratory testing including complete blood cell count, basic metabolic panel, liver function tests, testosterone and dehydroepiandrosterone levels, and chest radiograph have been normal.2 Additional workup for viral triggers also has been negative.15 A case series of 29 patients with LPP and its variants, including GLPL, revealed positive antinuclear antibodies in 10% of patients and a thyroid disorder in 24% of patients, with Hashimoto thyroiditis being the most prevalent in 7% of cases.16 There may be a strong association between the comorbidities of thyroid dysfunction and GLPL, as documented in other studies.10,17 A case-control study by Mesinkovska et al17 revealed a considerable increase in the prevalence of thyroid gland disease among patients with LPP vs controls. Human leukocyte antigen DR1 was found in a familial case of GLPL,4 and a case of GLPL following hepatitis B vaccination also has been described.18

Graham-Little-Piccardi-Lassueur syndrome most likely is a T-cell mediated autoimmune condition associated with one or multiple unknown keratinocyte antigens. Autoantibodies to the inner centromere protein were identified in a case that was positive on direct immunofluorescence, which may provide more insight into the disease pathophysiology.13 Interestingly, a study comparing the concentrations of inflammatory cells in LPP and traction alopecia found an elevation in the ratio of Langerhans cells to T lymphocytes within the follicular inflammatory infiltrate of LPP.19

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