Variant Of Lichen Planopilaris Research Articles

Vitamin D deficiency among patients with lichen planopilaris or frontal fibrosing alopecia

To the Editor: Lichen planopilaris (LPP), a prototypical, lymphocytic, cicatricial alopecia, is an inflammatory condition that affects the stem cell region of the hair follicle, known as the bulge. It may present as scalp alopecia; perifollicular scale and erythema; bald patches; and scalp pain, burning, or itching. Frontal fibrosing alopecia (FFA) is a variant of LPP, sharing some underlying pathology but differing in clinical presentation. LPP preferentially affects the vertex and crown scalp, whereas FFA primarily causes hair loss in the frontal hairline, eyebrows, and sideburns.

Characterization and treatment of prominent facial vasculature in the setting of frontal fibrosing alopecia

To the Editor: Frontal fibrosing alopecia (FFA) is a progressive scarring alopecia that is considered a variant of lichen planopilaris.1 It has been recently reported that the prominence of facial veins can be an initial presenting sign in many patients with FFA.2 However, the characterization of these patients has been limited. Long-pulse 1064-nm neodymium-doped yttrium aluminum garnet (Nd:YAG) lasers have been widely used for leg vein treatment and could serve as a similarly safe and effective treatment for prominent facial vasculature in the setting of FFA.

Platelet Rich Plasma in the Treatment of Frontal Fibrosing Alopecia

Frontal Fibrosing Alopecia (FFA) represents a form of scaring alopecia more frequent in postmenopausal women that presents with frontal hairline recession. It is typically classified as a variant of lichen planopilaris. Treatment of FFA can be challenging with poor long-term outcomes. Platelet-Rich Plasma (PRP) consists of an autologous concentration of platelets in a small volume of plasma. Activated platelets secrete cytokines and growth factors and thus may have a potential role in the treatment of inflammatory scarring alopecia such as FFA. A 68-year-old female with multi-resistant FFA was treated with lesional PRP injections every 4 weeks for 16 weeks. Baseline LPPAI score and phototrichograms targeting a representative area of disease activity were compared at baseline and at 16 weeks. After 16 weeks, no significant change in follicular units or follicular density from baseline to week 16 was noted. Only a minimal improvement in inflammatory activity observed clinically and through the Lichen Planopilaris Activity Index was observed. The discordance between the follicular density count and observed inflammatory activity suggests a longer treatment and observational period is needed. Additionally, the frequency of PRP injections potentially may also need to be increased. Given the limited efficacy of current therapies for FFA, PRP injections may be an option in patients with refractory disease, as an adjunct to systemic therapy. Additional investigation is needed to optimize frequency of PRP injections in FFA and to better assess its true anti-inflammatory effect.

5-Alpha-Reductase Inhibitor Treatment for Frontal Fibrosing Alopecia:A Myth or Truth?

Frontal Fibrosing Alopecia (FFA) is a condition described in 1994 affect mainly postmenopausal women. It is the most common form of cicatricial alopecia considered as a follicular variant of lichen planopilaris [1]. It is believed that the main pathogenesis in this condition is related to dihydrotestosterone, a derivative converted from the testosterone through 5-Alpha- Reductase (5AR). Because inflammatory, autoimmune, and other organisms are thought to be associated with FFA, there is no one specific cause that can be acted on by 5AR inhibitors [2].Currently, the therapeutic approaches are limited and the patient outcomes are poor [1]. To date, most of the reports are case reports and observational studies [3]. No randomized controlled studies are evaluating the pharmacodynamic profiles of 5AR inhibitors for FFA. The long-term safety of 5AR inhibitors in women should also be considered.

Frontal Fibrosing Alopecia - a review and a practical guide for clinicians.

Despite a significant increase in reported cases of frontal fibrosing alopecia (FFA) in literature, discussion about the possible role of environmental factors, instruction for diagnosis and guideline for treatment, are limited. The review aims to provide a detailed synthesis of this condition that could be used by clinicians in their practise. Whether single-centre or multi-centre, studies of more than 60 cases less than 5 years old were mainly taken into consideration. Results obtained were that FFA affects mainly postmenopausal Caucasian women; the most common comorbidities are hyperlipidaemia, arterial hypertension, osteoporosis, hypothyroidism, depression, alongside dermatological disorders such as atopic dermatitis, rosacea, seborrheic dermatitis and androgenetic alopecia. Autoimmune, genetic, hormonal (e.g. estrogen deficiency, pregnancy, lactation, HRT and raloxifene) and environmental (e.g. daily use of facial sunscreens and less frequent use of hair dyes and shampoo) hypotheses were proposed for pathogenesis, as well as association with various predisposing factors (patient's health-social profile, disease's history and comorbidities). Clinical presentation of FFA can be divided into 3 specific patterns, each with a different prognosis. Diagnosis is usually made clinically with the use of trichoscopy; however, scalp biopsy remains the gold standard. The condition is regarded as a variant of lichen planopilaris (LPP) due to the similarity of the prominent histopathological findings, but the clinical image is distinct and therapeutic options vary. 5α-reductase inhibitors, intralesional steroids, and hydroxychloroquine provide the highest level of evidence for the treatment of FFA. The conclusion is that a better understanding of the disease is crucial for proper disease management.

Clinical features and sex hormone profile in male patients with frontal fibrosing alopecia: A multicenter retrospective study with 33 patients

Clinical features and sex hormone profile in male patients with frontal fibrosing alopecia: A multicenter retrospective study with 33 patients

16899 Use of facial care products and frontal fibrosing alopecia: Is it coincidence or true association?

Background: Frontal fibrosing alopecia (FFA) is a variant of lichen planopilaris which usually involves the frontal hairline and sideburns. The increasing incidence of FFA over the last few decades suggests that environmental factors may be related to the pathogenesis. Recently, there are few studies reported the potential association between the use of facial care products and FFA.

Graham-Little-Piccardi-Lassueur Syndrome: Two Case Reports and Review of the Literature

Abstract Graham-Little-Piccardi-Lassueur syndrome (GLPLS) is a rare syndrome characterized by the triad of cicatricial alopecia of the scalp, non-cicatricial alopecia of the axilla and groin and follicular lichen planus eruptions on the trunk and extremities. GLPLS is considered to be a variant of lichen planopilaris. We report two cases that have fulfilled all of the criteria for GLPLS. The first case was a 71-year-old woman, admitted to the Department of Dermatology for pruritic perifollicullar erythema and scaling of the scalp, cicatricial scalp alopecia and hair loss of the axilla and pubic region for five months. Subsequently, follicular hyperkeratotic eruptions and hyperpigmented macules on the skin of the chest and abdomen appeared. The second case was a 48-year-old man with pruritic follicular papules on the face, trunk and extremities for four months. All of the laboratory examinations in both patients were within normal limits. No alternation in the general condition of the patients was observed. Histological examinations in both patients confirmed the diagnosis GLPLS. The patients were treated with systemic and local corticosteroid resulting in marked improvement of the skin lesions; however, cicatricial scalp alopecia showed no response to the treatment.

Using biopsies to study the differences between two hair loss disorders: lichen planopilaris and frontal fibrosing alopecia

British Journal of DermatologyVolume 183, Issue 3 p. e77-e77 Plain Language SummaryFree Access Using biopsies to study the differences between two hair loss disorders: lichen planopilaris and frontal fibrosing alopecia First published: 02 September 2020 https://doi.org/10.1111/bjd.19363AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinked InRedditWechat Abstract Lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are two uncommon disorders that both cause permanent, scarring alopecia (hair loss) and inflammation of the hair follicles. LPP affects both sexes and typically causes patchy alopecia over the central scalp, whereas FFA generally affects women and causes the hairline at the front of the scalp to recede. It can also cause eyebrow and body hair loss. Despite these distinct phenotypes (physical characteristics), FFA has traditionally been regarded as a variant of LPP. The authors sought to distinguish the two conditions using a wide array of markers for different cell types around the hair follicles. Markers are substances in the body, such as certain proteins, which act as signs to show, for example, the presence of certain cells or inflammation. The authors took skin punch biopsies (a type of tissue sample) from affected areas of scalp in patients with a diagnosis of LPP or FFA, and from healthy controls (people without LPP or FFA). In this study, the authors present a detailed, systematic analysis of a range of key inflammatory cell markers in LPP, FFA and controls. There were many similarities in the numbers and type of inflammatory cells in the two conditions. The study focussed on the different types of cells called macrophages seen in LPP and FFA. Broadly, these cells can be divided into two types, M1 cells, associated with inflammation and cell death, and M2 cells, involved in tissue repair. The two conditions differed in that more markers for M2 cells were seen in LPP in comparison to FFA. The authors hope that therapies directed against specific macrophage cell types may be developed in the future, preventing irreversible hair loss. Linked Article: Harries et al. Br J Dermatol 2020; 183:537–547. Volume183, Issue3September 2020Pages e77-e77 RelatedInformation

Profiling the human hair follicle immune system in lichen planopilaris and frontal fibrosing alopecia: can macrophage polarization differentiate these two conditions microscopically?

Frontal fibrosing alopecia (FFA) is traditionally regarded as a variant of lichen planopilaris (LPP) based on histological features. Distinct clinical presentation, demographics and epidemiology suggest that differing pathogenic factors determine the final phenotype. To map the hair follicle immune system in LPP and FFA by systematically comparing key inflammatory markers in defined hair follicle compartments. Lesional scalp biopsies from LPP and FFA and healthy controls were stained with the following immunohistochemical markers: CD1a and CD209, CD4, CD8, CD56, CD68, CD123, CXCR3, forkhead box (FOX)P3, mast cell tryptase and cKit. Macrophage polarization was explored using CD206, CD163, CD86, receptor for advanced glycation end products (RAGE), interleukin (IL)-4 and IL-13 on paired lesional and nonlesional LPP and FFA samples. Increased numbers of CD8+ , CXCR3+ and FOXP3+ T cells and CD68+ macrophages were identified in the distal hair follicle epithelium and perifollicular mesenchyme in both LPP and FFA compared with controls. In both LPP and FFA, total and degranulated mast cells and CD123+ plasmacytoid dendritic cells were increased in the perifollicular mesenchyme adjacent to the bulge and infundibulum, whereas numbers of CD1a+ and CD209+ dendritic cells were significantly reduced in the infundibulum connective tissue sheath. However, only with CD68 staining was a significant difference between LPP and FFA identified, with greater numbers of CD68+ cells in LPP samples. Furthermore, the identified macrophage polarization markers downregulated CD86 and upregulated CD163 and IL-4 expression in lesional LPP compared with FFA samples. This comparative immunopathological analysis is the first to profile systematically the hair follicle immune system in LPP and FFA. Our analysis highlights a potential role of macrophages in disease pathobiology and suggests that macrophage polarization may differ between LPP and FFA, allowing microscopic differentiation. Linked Comment:Kinoshita-Ise. Br J Dermatol 2020; 183:419-420.

Frontal fibrosing alopecia: An update on the hypothesis of pathogenesis and treatment.

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.

Algorithmic approach to the treatment of frontal fibrosing alopecia: A systematic review

To the Editor: Frontal fibrosing alopecia (FFA) is a lymphocytic primary cicatricial alopecia initially described by Kossard in 1994.1Vañó-Galván S. Molina-Ruiz A.M. Serrano-Falcón C. et al.Frontal fibrosing alopecia: a multicenter review of 355 patients.J Am Acad Dermatol. 2014; 70: 670-678Abstract Full Text Full Text PDF PubMed Scopus (277) Google Scholar FFA most commonly affects postmenopausal women, although cases in premenopausal women and men have been described. FFA is characterized by frontotemporal hairline recession, eyebrow hair loss, and perifollicular erythema and hyperkeratosis. There is no consensus for the treatment of FFA, and current therapy is aimed at preventing the progression of hair loss. Although some consider FFA to be a variant of lichen planopilaris, there might be treatment considerations unique to FFA. The authors have conducted a systematic review of the current literature to help in the development of a treatment algorithm for patients with FFA. On January 17, 2018, we conducted a search of the PubMed database using the term “frontal fibrosing alopecia treatment.” Publications not in English and those not including specific treatment outcomes were eliminated. A total of 22 studies were included in our review and are summarized in Supplementary Table I, available via Mendeley at https://doi.org/10.17632/nkt4k79spw.1. We utilized the levels of evidence tool from the Oxford Centre for Evidence-Based Medicine to assign grades of recommendation to each study. Treatment response was most often described as disease improvement, stabilization, or progression by authors of the original articles. Treatment regimens for 816 patients were described in the 22 studies. Topical and intralesional corticosteroids are often first-line therapies due to their minimal side effect profile and were most often used in the reviewed studies. These therapies were rarely used as solitary treatments, making it difficult to gauge their efficacy. In our review, hydroxychloroquine and 5a-reductase inhibitor (5aRIs; dutasteride and finasteride) were the most effective therapies. Sixty of 83 (72%) patients treated with hydroxychloroquine and 207 of 297 (70%) patients treated with 5aRIs experienced disease stabilization and improvement. The endpoint for most studies was disease stabilization, and in this regard, hydroxychloroquine and 5aRIs performed similarly. Patients typically experienced results within 6-12 months, although some continued to see improvement up to 2 years after treatment. Systemic corticosteroids were rarely used and were limited to cases of severe inflammation. Other therapies were utilized much less frequently with variable outcomes (Supplementary Table I). Despite decreased androgen levels noted in FFA patients, 5aRIs might nevertheless be an effective treatment option for these patients.2Ranasinghe G.C. Piliang M.P. Bergfeld W.F. Prevalence of hormonal and endocrine dysfunction in patients with lichen planopilaris (LPP): a retrospective data analysis of 168 patients.J Am Acad Dermatol. 2017; 76: 314-320Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar Use of 5aRIs should be limited to patients who are postmenopausal or on reliable contraception.3Donovan J.C. Finasteride-mediated hair regrowth and reversal of atrophy in a patient with frontal fibrosing alopecia.JAAD Case Rep. 2015; 1: 353-355Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar Given the findings of this review, we suggest the treatment approach for FFA outlined in Fig 1. In patients with continued disease progression despite standardized therapies, special consideration should be given to emerging therapies, such as platelet-rich plasma and low-level light-emitting diodes.4Fonda-Pascual P. Moreno-Arrones O.M. Saceda-Corralo D. et al.Effectiveness of low-level laser therapy in lichen planopilaris.J Am Acad Dermatol. 2018; 78: 1020-1023Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar, 5Dina Y. Aguh C. Use of platelet-rich plasma in cicatricial alopecia.Dermatol Surg. 2018; : 1https://doi.org/10.1097/DSS.0000000000001635Crossref PubMed Scopus (13) Google Scholar Limitations of this study include varying doses and lengths of treatments and subjective interpretation of disease progression and stabilization. Because there is currently no global index scale for FFA, it is difficult to assess the true efficacy of treatment. In addition, in some instances it was unclear whether resolution was due to the natural history of the disease or employment of a specific therapy.1Vañó-Galván S. Molina-Ruiz A.M. Serrano-Falcón C. et al.Frontal fibrosing alopecia: a multicenter review of 355 patients.J Am Acad Dermatol. 2014; 70: 670-678Abstract Full Text Full Text PDF PubMed Scopus (277) Google Scholar Moving forward, randomized controlled trials are necessary to evaluate the effectiveness of various treatments of FFA.

1377 Decoding frontal fibrosing alopecia and lichen planopilaris: Macrophages hold the key?

Frontal fibrosing alopecia (FFA) and Lichen planopilaris are inflammatory hair discorders leading to hair follicle (HF) destruction and scarring. Traditionally FFA has been regarded as a variant of lichen planopilaris (LPP) based on histological features, however, distinct phenotypic differences argue against this. So far clinicians and scientists have failed to describe the differences between each pathology. As macrophages are known scavengars of the immune system, having a role in programmed organ deletion and also are implicated in human hair cycle cotrol, they may hold the key. Therefore in this strudy we have investigated the macrophage populations utilising CD68 staining to investigate their role in LPP and FFA pathogenesis. Results showed that CD68 was expressed in both LPP and FFA compared with controls with staining being prominent in partially destroyed HFs. Comparing LPP to FFA, there was a significantly increased number of CD68+ cells in LPP HFs compared with both FFA and control samples suggesting they may have a different role in LPP when compared with FFA. This was found in both in the peri-follicular and the inter-follicular mesenchye. That the macrophage population was more prominent in LPP suggests they may have a more substantial role in the pathogenesis of LPP than FFA. Next it is essential to characterize the polarization of macrophages and the role these immune cells have in pathogenesis, i.e. a scavenger role or secretory cells producing cytokines such and IFN leading to IP loss and stem cell destruction. Our preliminary study provides the first evidence suggesting a subtle yet important difference in the pathogenesis of LPP and FFA. By understnding the differences between each condition we can aim to devise better targetted treatment strategies to prevent and restore hair loss leading to better clinical outcomes.

Frontal Fibrosing Alopecia: Update and Review of Challenges and Successes.

Frontal fibrosing alopecia (FFA) is a variant of lichen planopilaris (LPP) and is characterised as a progressive cicatricial alopecia affecting the frontotemporal hairline. To perform a comprehensive, up-to-date review of the etiopathogenesis, clinicopathological features, and therapeutic options for FFA. A literature search was conducted using PubMed (from 1946) and Cochrane (from 1991) databases on March 7, 2017. We included all retrospective and prospective studies reported in English. Only cases studies with reported treatment regimen and outcome were included. No randomised control trials were found. MeSH terms used included frontal fibrosing alopecia, postmenopausal, histopathologic, cicatricial, and treatment. With an increasing incidence of FFA occurring predominantly in postmenopausal women, progress has been made clinically and histologically in understanding this scarring alopecia. Conflicting results have been reported with various treatments, including intralesional or oral corticosteroids, antiandrogens, antimalarials, antibiotics, and surgery. To date, no randomised control trials for treatment of FFA have been conducted. The aetiology and clinical course of FFA remain to be established. Unfortunately, despite the numerous treatment options available, no one therapeutic regimen has proven effective in stopping recession of the hairline and inducing hair growth.

Frontal Fibrosing Alopecia

Frontal fibrosing alopecia, described simply over two decades in the past, has come to be probably the most normally noticeable causes of scarring phalacrosis at several skilled hair clinics. Regarded a medical variant of lichen planopilaris (LPP), its uncommon components and associations that distinguish it from LPP. Despite the fact that largely affecting post-menopausal ladies, a small however growing number of guys and premenopausal ladies are affected. The spectrum of the ailment has accelerated from involvement of the frontal hairline and eyebrows, to potentially affecting the whole hairline, facial and body hair. Genetic and environmental motives were implicated however the aetiology remains uncertain. A range of cures had been used in management of the, however medical trials are required to set up effectiveness.

Frontal fibrosing alopecia and lichen planopilaris in HLA-identical mother and daughter

Frontal fibrosing alopecia (FFA) is a lymphocyte-mediated scarring alopecia thought to be a variant of lichen planopilaris (LPP). We present a 67-year-old woman with frontal fibrosing alopecia whose daughter was diagnosed to have lichen planopilaris. Both patients had identical human leukocyte antigen (HLA) D types, supporting a phenotypical relationship between the two clinical entities. Interestingly, our patient also had of autoimmune chronic atrophic gastritis, a previously unreported association.

Male frontal fibrosing alopecia with generalised hair loss

Frontal fibrosing alopecia predominantly affects postmenopausal women and is regarded as a variant of lichen planopilaris. Male cases have rarely been reported. Here we describe a 66-year-old man with a typical receding fronto-temporal hair line in a form of scarring alopecia, which shows features of lichen planopilaris in histology. An extensive loss of body hair involving bilateral axillae, limbs and pubic area was also observed.

Lichen planopilaris following hair transplantation and face-lift surgery

Cosmetic surgical procedures, including hair transplantation and face-lift surgery, are becoming increasingly popular. However, there is very little information regarding the associated development of dermatological conditions following these procedures. Lichen planopilaris (LPP) is an uncommon inflammatory hair disorder of unknown aetiology that results in permanent alopecia and replacement of hair follicles with scar-like fibrous tissue. Frontal fibrosing alopecia (FFA), a variant of LPP, involves the frontal hairline and shares similar histological findings with those of LPP. We report 10 patients who developed LPP/FFA following cosmetic scalp surgery. Seven patients developed LPP following hair transplantation, and three patients developed FFA following face-lift surgery. In all cases there was no previous history of LPP or FFA. There is currently a lack of evidence to link the procedures of hair transplantation and cosmetic face-lift surgery to LPP and FFA, respectively. This is the first case series to describe this connection and to postulate the possible pathological processes underlying the clinical observation. Explanations include Koebner phenomenon induced by surgical trauma, an autoimmune process targeting an (as yet, unknown) hair follicle antigen liberated during surgery or perhaps a postsurgery proinflammatory milieu inducing hair follicle immune privilege collapse and follicular damage in susceptible individuals.

Frontal fibrosing alopecia associated with generalized hair loss

We present six cases of frontal fibrosing alopecia, in which generalized hair loss is a feature. Although this variant of lichen planopilaris has been reported clinically in a number of patients, there is very little histological evidence that the condition exists in peripheral sites. We believe this pattern of involvement may be more common than is reported, and have provided histological evidence of lichen planopilaris being present at sites beyond the scalp and eyebrows.

Alopecia frontal fibrosante: relato de seis casos

Alopecia frontal fibrosante é forma progressiva de alopecia cicatricial. Os casos iniciais foram relatados a partir 1994, na Austrália, em pacientes do sexo feminino pós-menopausa. Desde então inúmeros casos foram descritos na literatura sugerindo que ela é mais prevalente do que inicialmente se supunha. Seu curso progressivo se assemelha ao da alopecia androgenética; histologicamente, entretanto, o infiltrado liquenóide é evidente. O artigo relata seis casos brasileiros e discute a alopecia frontal fibrosante dentro do grupo das alopecias cicatriciais, como variante do líquen plano pilar.