Lichen planopilaris (LPP) is an immune-mediated cicatricial alopecia. The main clinical presentations of LPP include classic form, frontal fibrosing alopecia (FFA), and Graham-Little-Piccardi-Lassueur syndrome (GLPLS). We reviewed medical records of all 291 patients diagnosied with LPP from 2006 to 2017 in Department of Dermatology, Tehran University of Medical Sciences. LPP was more common in women than men. Lichen planus (LP) was seen in 59 of patients (20.3%). Parietal lesions (69.75%), frontal (27.14%), occipital (23.71%), and temporal (21.64%) were frequently seen in LPP patients. However, trunk hair involvement (15.4% vs. 2.7%; P-value = 0.011) and eyebrow involvement (57.7% vs. 0%; P-value < 0.0001) were high in FFA patients. Patients treated with cyclosporine (CSP) achieved partial response (PR) and complete response (CR) faster than with methotrexate (MTX) and mycophenolate mofetil (MMF, P-value=0.0001). So,, CSP and MTX can be considered as effective alternative treatments for non-responders. Moreover, MTX was more effective than MMF but not different in time to reach PR (P-value=0.23) or CR (P-value=0.56). However, CSP and MTX were less safe compared with MMF. 5-alpha reductase inhibitors, systemic retinoids (isotretinoin) or their combination were the most effective therpeutic options for FFA patients.

Frontal fibrosing alopecia (FFA) is a relatively new scarring alopecia that is considered a variant of lichen planopilaris (LPP) with no recognized promising treatments. In this study, we tried to clarify the underlying signaling pathways and their roles in the pathogenesis and progression of FFA. Because of several differences in clinical manifestations, response to treatments, and pathological findings, these two conditions could be differentiated from each other. Taking into account the already discussed signaling pathways and involved players such as T cells, mast cells, and sebaceous glands, different possible therapeutic options could be suggested. In addition to treatments supported by clinical evidence, such as 5 alpha-reductase inhibitors, topical calcineurin inhibitors, hydroxychloroquine, peroxisome proliferator-activated receptor gamma agonists, and oral retinoid agents, various other treatment strategies and drugs, such as phototherapy, Janus kinase inhibitors, dehydroepiandrosterone, sirolimus, cetirizine, and rituximab, could be suggested to mitigate disease progression. Of course, such lines of treatment need further evaluation in clinical trials.