Discussion
G raham-Little-Piccardi-Lassueur syndrome GLPLS 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 3. Around 50 cases of GLPLS have been reported since then4.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 follicular papule over body. Its cause remains unknown, but more likely is a T-cell mediated autoimmune condition 4. Recent studies showed that there is decrease expression of peroxisome proliferator-activated receptor (PPAR) and many patients respond well to PPARγ agonists 5.also, interferon and JAK singling is upregulated in LPP6.
The goal of treatment in GLPLS as well as in other scarring alopecia is to prevent progression of hair loss thus early diagnosis and intervention is crucial 1. Many treatment modalities have been used in treating lichen planopilaris with variable results. Treatment options range from topical and intralesional steroid to systemic treatment such as hydroxychloroquine, cyclosporine and pioglitazone7. Baibergenova and Walsh8 used PPARγ agonists (Pioglitazone) which induced complete remission in 25% and significantly improved symptoms in 50% of patient diagnosed to have LPP. Pioglitazone side effects are very mild including calf pain, lightheadedness, nausea, dizziness, and hives which were experienced by less than 5% of patients8. Chiang et al 9 studied the use of hydroxychloroquine in the treatment of LPP in 40 patients for twelve months. Their results showed that hydroxychloroquine was very effective in terms of controlling symptoms and halting disease progression with a 69% and 83% significant reduction in severity of LPP at both 6 and 12 months respectively. Treatment with oral tofacitinib either as monotherapy or as adjuvant to other treatment showed measurable 80% improvement clinically6.Excimer laser(308-nm) was used by Navarini et al twice weekly in 13 patients and all patient experienced relief of pruritus with 40% reduction in inflammation but only 25% of patients had hair regrowth10. Finally, naltrexone was used and showed improvement mainly in term of relieving symptoms such as pruritus11. Our patient was started on hydroxychloroquine after he was evaluated by ophthalmology and there was no contraindication to start the medication. In addition, the patient was started on topical treatment in the form of tretinoin 0.05% cream targeting follicular keratotic papules. On follow up, the patient reported improvement in term of pruritis and reduction on the severity of follicular keratotic papules.