Graham-Little-Piccardi-Lasseur Syndrome Workup
- Author: Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada); Chief Editor: Dirk M Elston, MD more...
Some epidemiological studies describe a loose association between mucocutaneous lichen planus and hepatitis C. However, this association has not yet been described in case reports of Graham-Little-Piccardi-Lasseur syndrome (GLPLS).
Initial investigations may include antinuclear antibodies (ANA), antiextractable nuclear antigens (AENA), hepatitis B and C serology, and liver function tests to exclude other associated systemic causes of cicatricial alopecia.
No imaging studies are required for Graham-Little-Piccardi-Lasseur syndrome (GLPLS).
Punch biopsies of the scalp parallel to hair shaft growth (or directed at 45° angle in African Americans) may confirm the presence of cicatricial alopecia. Multiple biopsy samples may be required for transverse (horizontal) and vertical sections, and immunohistochemistry. Histologically, the end-stage lesions in Graham-Little-Piccardi-Lasseur syndrome (GLPLS) are indistinguishable from those of pseudopelade of Brocq, discoid lupus erythematosus, folliculitis decalvans, frontal fibrosing alopecia, and other forms of cicatricial alopecia.
A skin biopsy of associated follicular papules may reveal the presence of histologic findings that correlate with the triad of GLPLS.
The presence of noncicatricial alopecia in the axilla and groin can usually be diagnosed clinically, and further skin biopsies are not necessary.
In the early stage, an inflammatory perifollicular lichenoid infiltrate can be observed and is often associated with the infundibuloisthmic (bulge) region of the follicle. This infundibuloisthmic region contains stem cells responsible for regenerating the lower two thirds of the hair follicle, which is nonpermanent. The bulb region is spared. In end-stage Graham-Little-Piccardi-Lasseur syndrome (GLPLS), atrophic dermis and fibrosed and empty hair shafts can be seen. One additional histological finding is keratinous follicular plugs with loss of sebaceous glands.
A lichenoid lymphocytic infiltrate composed of mostly CD8 and CD4 T cells may irreversibly damage stem cells and hair follicles. Profibrogenic cytokines such as interleukin (IL)–4 and IL-6, transforming growth factor (TGF)–beta, and interferon (IFN)–gamma have been reported in association with lichen planopilaris of the scalp.
Direct immunofluorescence studies have reported nonspecific immunoglobulin M, and occasionally immunoglobulin G and immunoglobulin A, at the hair follicle infundibulum and isthmus, as well as linear fibrinogen deposition along the dermoepidermal junction.
Histopathology of follicular papules
Findings include (1) lichenoid lymphocytic infiltrate in the upper dermis, (2) hyperkeratosis and focal hypergranulosis, (3) acanthosis with occasional saw-toothed rete ridges, and (4) dyskeratotic keratinocytes (Civatte or colloid bodies).
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