ArticlePDF Available

It is not invisible! A case report of 2 patients with scalp Lichen Planopilaris mimicking Androgenic Alopecia

  • Giuliani, Milan, Italy
  • Private Office , Milan, Italy
  • International Hair Research Foundation

Abstract and Figures

Lichen Planopilaris is known as the form of Lichen Planus typical of the scalp. It is classifi ed as a lymphatic disease and is characterized by chronic infl ammation which leads to cicatricial alopecia. Its causes are not yet well characterized but its etiology seems to strongly correlates with infection, sensitization and pollution. A clear and objective diagnosis of Lichen Planopilaris is not simple but the evolution and strongly negative outcomes on scalp of people affected by, pose the need of an early diagnosis. In this work we report the case of a 27-year-old male and a 54-year-old female, respectively, in which a correct diagnosis of Lichen Planopilaris, followed the incorrect previous ones, was made by means of dermatoscopy and histopathological analysis, decisive tools for the diagnosis of this kind of pathology.
Content may be subject to copyright.
Annals of Dermatological Research
Open Access
Lichen Planopilaris is known as the form of Lichen Planus typical of the scalp. It is classi ed as a lymphatic
disease and is characterized by chronic in ammation which leads to cicatricial alopecia. Its causes are not yet
well characterized but its etiology seems to strongly correlates with infection, sensitization and pollution. A clear
and objective diagnosis of Lichen Planopilaris is not simple but the evolution and strongly negative outcomes on
scalp of people affected by, pose the need of an early diagnosis.
In this work we report the case of a 27-year-old male and a 54-year-old female, respectively, in which a correct
diagnosis of Lichen Planopilaris, followed the incorrect previous ones, was made by means of dermatoscopy
and histopathological analysis, decisive tools for the diagnosis of this kind of pathology.
Case Report
It is not invisible! A case report of 2
patients with scalp Lichen Planopilaris
mimicking Androgenic Alopecia
Sorbellini Elisabetta1, Pinto Daniela2, Marzani Barbara2 and
Fabio Rinaldi1*
1Studio Rinaldi & Associati, Milan, Italy
2International Hair Research Foundation (IHRF), Milan, Italy
*Address for Correspondence: Fabio Rinaldi,
Studio Rinaldi & Associati, Milan, Italy, Tel.: +39-2-
76006089; Email:
Submitted: 24 November 2017
Approved: 06 December 2017
Published: 08 December 2017
Copyright: 2017 Sorbellini E, et al. This is an
open access article distributed under the Creative
Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly
Abbreviations: LP: Lichen Planus; LPP: Lichen
Planopilaris; FFA: Frontal Fibrosing Alopecia;
FAPD: Fibrosing Alopecia in a Pattern Distribution;
VAS: Visual Analogue Scale; AGA: Androgenetic
How to cite this article: Sorbellini E, Pinto D, Marzani B, Rinaldi F. It is not invisible! A case report of 2 patients with scalp
Lichen Planopilaris mimicking Androgenic Alopecia. Ann Dermatol Res. 2017; 1: 012-017.
Lichen planopilaris (LPP), a morphological variant of Lichen Planus known as
speciic of the scalp, is a rare, dificult-to-treat, chronic, inlammatory skin condition
resulting in cicatricial alopecia, which can be eventually permanent irreversible [1]. It
has been described for the irst time by Pringle in 1895 [2] and placed in the lymphocytic
disease group by the North American Hair Research Society (NAHRS) [3]. The causes
of this disease are not well clariied but it seems to act as an autoimmune disorder
probably triggered by drugs, metal exposure, stress, pollution, contacts sensitizers
or infection [4]. Epidemiological data report that about 0.5-1% of the population is
affected by Lichen Planus and in particular, adult women result as the most affected
population by LPP (ratio 1.8:1) [5,7], on people aged between 40-60 years and more
frequently in Caucasian and Indian population than Asian ones [7,8] Its typical
manifestation is multifocal areas of alopecia on the central scalp, usually accompanied
by perifollicular erythema, hyperkeratosis, and subjective symptoms such as pruritus
or pain [9]. Three different variants of LPP are normally recognized [3,9]: classic LPP,
frontal ibrosing alopecia (FFA) [10] and Graham-Little Piccardi Lassueur Syndrome
[11]. A fourth variant, known as ibrosing alopecia in a pattern distribution (FAPD)
could be added if considering some cases of patients with androgenetic alopecia in
which lesions identical to those seen in LPP were found [12].
The main therapy for LPP is topical treatment with class I or II, high-potency topical
corticosteroids such as clobetasol propionate or intralesional triamcinolone injection
[13]. Some other topical therapeutics approaches can include calcineurin inhibitors
(cyclosporine, tacrolimus), even if limited data are reported. More recently also the use
of mycophenolate mofetil has been proposed [13-16]. Driven by recent evidences on
the role of peroxisome-proliferator-activated receptor (PPAR)-γ on the pathogenesis of
It is not invisible! A case report of 2 patients with scalp Lichen Planopilaris mimicking Androgenic Alopecia
Published: December 08, 2017 13/17
LPP [17], also PPAR-γ antagonist, pioglitazone, has been recently included as effective
therapy. In presence of severe not responsive cases, systemic and/or intralesional
costicosteroids have been largely recommended [6] and tipically used in combination
with topical corticosteroid treatment, with limitations as regards side-effects.
As typical when referring to scalp disease to make a clear and objective diagnosis is
not easy. LPP manifestations evolve with the disease: early LPP can be easily confused
with seborrheic dermatitis, on the other side, in the late stages, LPP can be confused
with central centrifugal cicatricial alopecia (CCCA) and folliculitis decalvans [1]. The
approach of differential diagnosis reveals to be crucial in order to counteract the
progressive and chronic course of the pathology and to establish the correct therapy
as well as the use of dermatoscopy and histological evaluation in condition in which
LPP could result invisible at the clinical evaluation.
Case Report
Case report 1
A 27-year-old male (Milan, Italy), outdoor worker, reported a history of scalp
thinning and hair loss for 1 year. Other symptoms reported were moderate pruritus,
≥4 according to VAS scale [18], erythema and scaling. No evidence of Lichen Planus in
other body sites. All these symptoms were evaluated by the previous dermatologist
as compliant with androgenetic alopecia associated with seborrheic dermatitis.
Consequently, the patient was invited to treatment with Minoxidil 5% solution for
three months. The therapy was then suspended because of erythema and itching
worsening. Patient came to our examination because in his opinion hair loss and
symptoms did not disappear. Physical examination of scalp conirmed erythema and
localized thinning both in vertex and frontal area (Figure 1).
The area affected was also analyzed by a Dermatoscope with a 100x polarized
magniication (Molemax HD, DermaInstrument, Vienna, Austria). The Molemax
integrated software was used for images acquiring (Figure 2). Dermatoscopic
examination conirmed the presence of erythema and scalp scaling mainly at
perifollicular site but reported also the absence of signiicant miniaturization, which
is typical of androgenetic alopecia. Dermatoscopy showed also the presence of tubular
casts at the perifollicular level. Taking together the history of the patient and the latest
evaluations, a preliminary diagnosis of LPP was made, subsequently conirmed by
histopathological evaluation.
The analysis of histological section highlighted reduction in epidermis thickness,
presence of skin sclerosis, vasodilatation, melanin pigment residues in the supericial
dermis, vertical ibrotic bands and residues of pre-existing hair follicles (Figure 3). A
dense peripheral lymphocytic iniltration was also found. All these observations were
clearly consistent with LPP.
Figure 1: Localized scaling on vertex and parietal/mild frontal area of case report 1.
It is not invisible! A case report of 2 patients with scalp Lichen Planopilaris mimicking Androgenic Alopecia
Published: December 08, 2017 14/17
Case report 2
A 54-year-old female came to our clinic reporting a history of therapy for telogen
efluvium 2 years before. Patient went through menopause three years before and
reported important symptoms such as sever osteoporosis, hot lush, insomnia, mood
swings. Any hormone replacement therapy has been suggested to the patient because
breast cancer familiarity.
Clinical evaluation showed scalp thinning both in vertex and frontal area (Figure 4).
Patient referred also a persistent trichodinia. Also in this case no evidence of Lichen
Planus in other body sites were found.
Patient anamnesis and clinical evaluation suggested the dermatologist to proceed
further with dermatoscopic analysis. Images observation (Figure 5) revealed, among
the rest, arborized vasodilatation, which is a typical manifestation of LPP. This
diagnosis was inally conirmed by histology (Figure 6).
Tre a t m e n t
After LPP diagnosis, both patients were treated with clobetasol propionate foam
0.05% on affected area once a day for 30 days, following by 15 days of resting and
further 30 days of treatment once a day. Treatment succeeded into reduce inlammatory
symptoms, also alleviating erythema, itching and ibrosis too. This contribute to
stabilize LPP avoiding further evolution of the pathology as demonstrated by the
following histopathological analysis.
LPP is an uncommon scalp disease which diagnosis remains a challenge due to
epidemiological data lacking and especially to the increasing number of patients in
which lesions and symptoms are not clearly visible or evaluable if limited to patient
Figure 2: Dermatoscopic analysis on case report 1 showing perifollicular tubular casts (arrow).
Figure 3: Histopathological analysis on case report 1, showing melanin incontinence on super cial dermis (red
arrow), perifollicular in ltrates (asterisk).
It is not invisible! A case report of 2 patients with scalp Lichen Planopilaris mimicking Androgenic Alopecia
Published: December 08, 2017 15/17
clinical history and clinical observations. This led, in most cases, to a wrong diagnosis,
preferably of androgenetic alopecia (AGA) with which LPP shares some macroscopic
features. Some authors report these situations as “invisible LPP” [19,20]. The prognosis
for LPP is worse than AGA, evolving into bulge stem cells disruption and scarring
[6,21,22], leading to irreversible baldness. Therefore, LPP is often accompanied with
persistent trichodynia, a burning scalp syndrome strongly affecting quality of life of
patients, which are affected by [23]. From this the importance of an immediate and
correct diagnosis for reducing LPP discomfort and stopping pathology evolution by
early therapeutic intervention.
In the cases reported in the present work, we made a diagnosis of LPP, apparently
invisible and following a previous wrong diagnosis, by means of dermatoscopic
analysis coupled with histopathological examination. Patients were then submitted
to treatment with clobetasol foam and this led the inlammation to be stabilized and
at the same time hair loss stopping and trichodynia disappearance. Therapy with
Figure 4: Localized scaling on vertex of case report 1.
Figure 5: Dermatoscopic analysis on case report 2 showing perifollicular casts (arrow).
Figure 6: Histopathological analysis on case report 2.
It is not invisible! A case report of 2 patients with scalp Lichen Planopilaris mimicking Androgenic Alopecia
Published: December 08, 2017 16/17
topical corticosteroid with mid-to high-potency is generally preferred as irst-line the
treatment of LPP with a resolution of the patology higher than 66% [5,6,23] conirming
as the mainstay therapy for LPP.
According to our experience [24] LPP incidence is increasing with respect to
reported statistics and this, probably, due to the impact on the pathology of etiological
factors such as hair dying or microbioma dysbiosis [25]. There are also some studies
demonstrating the involvement of environment in skin pathology including LPP [26,
27]. Pollution may act as oxidant agent and induce oxidative stress by increasing the
production of reactive oxygen species (ROS) directly or indirectly [28]. LPP incidence
is growing also because an improvement in diagnostic by means of dermatoscopy
and histopathology that revel themselves as essential tools for make “visible” the
Informed consent
Written informed consent was obtained from the patient for the publication of this
case report and accompanying images.
Confl ict of interest
R.F. serves as a consultant for Giuliani S.p.A. P.D. and M.B. are employed by Giuliani
1. Assouly, Philippe; Reygagne, Pascal. Lichen planopilaris: update on diagnosis and treatment. Semin
Cutan Med Surg. 2009; 28: 3-10. Ref:
2. Pringle JJ. Lichen pilaris spinulosus. Br J Dermatol. 1905; 17: 78-97.
3. Olsen EA, Bergfeld WF, Cotsarelis G, Price V, Shapiro R, et al. Workshop on cicatricial alopecia.
Summary of North American Hair Research Society (NAHRS)-sponsored workshop on cicatricial
alopecia, Duke University Medical Center, February 10 and 11, 2001. J Am Acad Dermatol. 2003;
48:103-110. Ref:
4. Cooper S. Lichen planopilaris. Orphanet. 2011; Ref:
5. Chieregato C, Zini A, Barba A, Magnanini M, Rosina P. Lichen planopilaris: report of 30 cases and
review of the literature. Int J Dermatol. 2003; 42: 342-345. Review. Ref:
6. Nathaniel C. Cevasco, Wilma F. Bergfeld, Berna K. Remzi, Haydee Ramirez de Knott. A case-series
of 29 patients with lichen planopilaris: the Cleveland Clinic Foundation experience on evaluation,
diagnosis, and treatment. J Am Acad Dermatol. 2007; 57: 47-53. Ref:
7. Hoon Kang, Abdullateef A. Alzolibani, Nina Otberg, Jeery Shapiro. Lichen planopilaris. Dermatol Ther.
2008; 21: 249-256. Ref:
8. Lehman JS, Tollefson MM, Gibson LE. Lichen planus. Int J Dermatol. 2009; 48: 682-694. Ref:
9. Weston G, Payette M. Update on lichen planus and its clinical variants. Int J Womens Dermatol.
2015; 16:140-149. Ref:
10. Samrao A, Chew AL, Price V. Frontal brosing alopecia: a clinical review of 36 patients. Br J Dermatol.
2010; 163: 1296-1300. Ref:
11. Odom RB, James WD, Berger TG, editors. Andrews’ Diseases of the skin. 9th ed. Philadelphia:
WB Saunders Elsevier, Lichen planus and related Conditions; pp. 2006; 274-275. Ref:
12. Zinkernagel MS, Trüeb RM. Fibrosing alopecia in a pattern distribution: Patterned lichen planopilaris
or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch Dermatol. 2000; 136: 205-
211. Ref:
13. Whiting D. Cicatricial alopecia: clinico-pathological ndings and treatment. Clin Dermatol. 2001; 19:
211-225. Ref:
14. Mehregan DA, Van Hale HM, Muller SA. Lichen planopilaris: clinical and pathologic study of forty- ve
patients. J Am Acad Dermatol. 1992; 27: 935-942. Ref:
It is not invisible! A case report of 2 patients with scalp Lichen Planopilaris mimicking Androgenic Alopecia
Published: December 08, 2017 17/17
15. Tan E, Martinka M, Ball N, Shapiro J. Primary cicatricial alopecias: clinicopathology of 112 cases. J
Am Acad Dermatol. 2004; 50: 25-32. Ref:
16. Tursen U, Api H, Kaya T, Ikizoglu G. Treatment of lichen planopilaris with mycophenolate mofetil.
Dermatol Online J. 2004; 10: 24. Ref:
17. Mirmirani P, Karnik P. Lichen planopilaris treated with a peroxisome proliferator-activated receptor
gamma agonist. Arch Dermatol. 2009; 145: 1363-1366. Ref:
18. Reich A, Heisig M, Phan NQ, Taneda K, Takamori K, et al. Visual analogue scale: evaluation of
the instrument for the assessment of pruritus. Acta Derm Venereol. 2012; 92: 497-501. Ref:
19. Nirmal B, George R, Kodiatte TA. Invisible lichen planopilaris unmasked by dermatoscopy. Int J
Trichol. 2017; 9: 76-78. Ref:
20. Grupper MC, Buisson J, Durepaire R, Edelson Y. [Gougerot’s invisible lichen planus]. Bull Soc Fr
Dermatol Syphiligr. 1971; 78: 598-599. French. Ref:
21. d’Ovidio R, Sgarra C, Conserva A, Angelotti UF, Erriquez R, Foti C. Alterated integrin expression in
lichen planopilaris. Head Face Med. 2007; 3: 11. Ref:
22. Baquerizo Nole KL, Nusbaum B, Pinto GM, Miteva M. Lichen Planopilaris in the Androgenetic
Alopecia Area: A Pitfall for Hair Transplantation. Skin Appendage Disorders. 2015; 1: 49-53. Ref:
23. Chiang C, Sah D, Cho BK, Ochoa BE, Price VH. Hydroxychloroquine and lichen planopilaris: ef cacy
and introduction of Lichen Planopilaris Activity Index scoring system. J Am Acad Dermatol. 2010;
62: 387-392. Ref:
24. Sorbellini E, Pinto D, Marzani B, Rinaldi F. Considerations about Lichen Planopilaris and the correct
diagnosis. JPD. 2018 in press.
25. He Y, Gong D, Shi C, Shao F, Shi J, at. Dysbiosis of oral buccal mucosa microbiota in patients with
oral lichen planus. Oral Dis. 2017; 23: 674-682. Ref:
26. Srivastava K. Urbanization and mental health. Ind Psychiatry J. 2009; 18:75-76. Ref:
27. Dlova N, Goh CL, Tosti A. Familial frontal brosing alopecia. Br J Dermatol. 2012; 168: 220-2.Ref:
28. Bickers DR, Athar M. Oxidative stress in the pathogenesis of skin disease. J Invest Dermatol. 2006;
126: 2565-2575. Ref:
... Nowadays LPP diagnosis is based on clinical and histopathological findings and on an accurate differential diagnosis that mainly evidences the presence of seborrheic dermatitis, psoriasis and AGA at an initial stage [16]. Due also to poor information on physiopathology and a not fully understood etiology, a complete and accurate diagnosis of LPP remains an open challenge. ...
Full-text available
Lichen Planopilaris (LPP) is a lymphatic disease affecting the scalp that is characterized by a chronic and destructive inflammation process, named as ‘cicatricial alopecia’ in which the hair follicles are targeted and may involve predominantly lymphocytes or neutrophils. Scalp and biopsy layers have never been used to investigate microbial community composition and its relative taxa abundances in LPP. We sought to examine the significant taxa of this chronic relapsing inflammatory skin disease, together with inspect the existing connections with metabolic pathways featuring this microbial community. We used a multilevel analysis based on 16S rRNA marker sequencing in order to detect OTU abundances in pathologic/healthy samples, real time PCR for measuring the levels of IL-23 interleukin expression and urinary metabolomics to find out volatile organic metabolites (VOMs). By using a linear regression model, we described peculiar taxa that significantly differentiated LPP and healthy samples. We inspected taxa abundances and interleukin mRNA levels and the Microbacteriaceae family resulted negatively correlated with the IL-23 expression. Moreover, starting from 16S taxa abundances, we predicted the metabolic pathways featuring this microbial community. By inspecting microbial composition, sample richness, metabolomics profiles and the relative metabolic pathways in a cohort of LPP and healthy samples we deepened the contribution of significant taxa that are connected to inflammation maintenance and microbiota plasticity in LPP pathology.
Full-text available
Objective: The bacterial community structure of buccal mucosa in patients with oral lichen planus was evaluated and compared with healthy control. Subjects and methods: Buccal scraping samples have been taken on 43 oral lichen planus patients (21 erosive and 22 non-erosive) and 21 mucosal healthy volunteers. The V3 hypervariable 16S rDNA region were amplified and sequenced by high-throughput 454 pyrosequencing. Results: 94.26% of the total buccal bacteria were classified into 15 abundant genera. 8 of these abundant genera could be detected in all cases, namely: Streptococcus, Prevotella, Haemophilu, Neisseria, Fusobacterium, Leptotrichia, Veillonella and Actinomyces. Four abundant bacteria showed significantly different prevalence at the genus level: Streptococcus was more abundant (p<0.01) in healthy control group, while Fusobacterium (p<0.01), Leptotrichia (p<0.001) and Lautropia (p<0.001) showed higher abundance in OLP group. Few differences can be found between erosive and non-erosive OLP. In general, 19 "core" OTUs at 3 levels were defined to be OLP specific, comparing with healthy control group. Conclusions: These results suggest that OLP is associated with dysbiosis of the oral microbiome. Further studies should be taken to elucidate the inner relationship between these observed changes and OLP development. This article is protected by copyright. All rights reserved.
Full-text available
Lichen planus (LP) is an inflammatory skin condition with characteristic clinical and histopathological findings. Classic LP typically presents as pruritic, polygonal, violaceous flat-topped papules and plaques; many variants in morphology and location also exist, including oral, nail, linear, annular, atrophic, hypertrophic, inverse, eruptive, bullous, ulcerative, lichen planus pigmentosus, lichen planopilaris, vulvovaginal, actinic, lichen planus-lupus erythematosus overlap syndrome, and lichen planus pemphigoides. Clinical presentation of the rarer variant lesions may be largely dissimilar to classic LP and therefore difficult to diagnose based solely on clinical examination. However, histopathological examination of LP and LP-variant lesions reveal similar features, aiding in the proper diagnosis of the disease. Management of LP and LP variants aims to control symptoms and to decrease time from onset to resolution; it often involves topical corticosteroids, but varies depending on the severity and location of the lesion. The literature contains an array of reports on the variations in presentation and successful management of LP and its variants. A familiarity with LP and its variants is important in achieving timely recognition and management of the disease.
Full-text available
The aim of this study was to evaluate the visual analogue scale (VAS) as a method of pruritus assessment. A total of 310 subjects with pruritic dermatoses (148 Caucasian subjects and 162 Asian subjects) were recruited. The patients assessed pruritus intensity using the horizontal and vertical VAS, numeric rating scale (NRS) and verbal rating scale (VRS). All scales showed very good reproducibility (intraclass coefficient (ICC) > 0.8). No significant differences were found between the horizontal and vertical VAS (5.3 ± 2.9 vs. 5.3 ± 3.0 points, p = 0.34). Using NRS, patients rated their pruritus significantly higher than with VAS (5.7 ± 2.6 points, p < 0.01). VRS showed the highest correlation with NRS (R = 0.82, p < 0.001), followed by horizontal (R = 0.75, p < 0.001) and vertical VAS (R=0.74, p < 0.001). Based on detailed analysis following VAS categories were proposed: 0 = no pruritus, > 0-< 4 points = mild pruritus, ≥ 4 -< 7 points = moderate pruritus, ≥ 7-< 9 points = severe pruritus, and ≥ 9 points = very severe pruritus. In conclusion, the VAS is a valuable method of pruritus measurement.
Lichen planopilaris is a form of lymphocyte-mediated primary cicatricial alopecia characterized by perifollicular scaling progressing to patches of alopecia depending on the clinical variant. The course is relentlessly progressive and chronic. Hence, early diagnosis and institution of therapy are imperative to halt the disease progress. Although definitive diagnosis is made by scalp biopsy, the detection is usually delayed. Dermatoscopy helps in early recognition of this condition which at that stage is clinically invisible. We report a 23-year-old female who presented with hair loss and scalp scaling without clinically obvious patches of alopecia. © 2017 International Journal of Trichology Published by Wolters Kluwer - Medknow.
Background: Classic lichen planopilaris (LPP) is a patchy form of primary lymphocytic cicatricial alopecia localized on the vertex of the scalp. It is important, however, to be aware of other, less recognized presentations that may be missed without dermatoscopy and pathology. Methods and results: We report 26 patients with LPP presenting with subtle erythema and scaling colocalized in the area of patterned thinning (androgenetic alopecia, AGA). All patients had been treated for seborrheic dermatitis in the past. Dermatoscopy showed the presence of 2-4 hairs emerging as a tuft from the same ostium surrounded by erythema, peripilar casts and interfollicular scaling associated with hair miniaturization. Histopathology obtained from those areas corresponded to LPP with concomitant follicular miniaturization. Conclusion: Subtle or focal cases of LPP may be missed for seborrheic dermatitis when overlapping with AGA. Dermatoscopy-guided biopsy from the affected scalp is the best approach to make a timely diagnosis. This is particularly important in patients with AGA evaluated to undergo hair transplantation, as active LPP is a contraindication for these patients.
Background Androgenetic alopecia is characterized by a defined area of progressive nonscarring alopecia. The clinical and histological findings in 15 women and 4 men with progressive scarring alopecia in a pattern distribution were studied. The results were evaluated and compared with clinicopathologic entities that feature scarring of the central scalp area, specifically, lichen planopilaris, pseudopelade, and follicular degeneration syndrome.Observations Patients developed progressive fibrosing alopecia of the central scalp, without the multifocal areas of involvement typical of lichen planopilaris and pseudopelade. Perifollicular erythema, follicular keratosis, and loss of follicular orifices were limited to a patterned area of involvement. Biopsy specimens of early lesions demonstrated hair follicle miniaturization and a lichenoid inflammatory infiltrate targeting the upper follicle region. Advanced lesions showed perifollicular lamellar fibrosis and completely fibrosed follicular tracts indistinguishable from end-stage lichen planopilaris, pseudopelade, or follicular degeneration syndrome.Conclusions Some patients with androgenetic alopecia might have additional clinical and histological features of inflammation and fibrosis limited to the area of androgenetic hair loss. In these patients, the histological findings of early lesions are identical to those seen in lichen planopilaris. The lichenoid tissue reaction leading to follicular destruction in these patients might be pathogenetically related to the events underlying androgenetic alopecia.
Frontal fibrosing alopecia (FFA) is a cicatricial alopecia that was first described by Kossard in 1994 (1) . It primarily affects Caucasian postmenopausal women with progressive recession of the frontotemporal hairline .In recent years the disease has been increasingly reported in Europe (2,3,4,) US (5) and Japan.(6) Most authors consider FFA a variant of lichen-planopilaris(LPP) because of its similar pathological features .
Frontal fibrosing alopecia (FFA) is a primary lymphocytic cicatricial alopecia with a distinctive clinical pattern of progressive frontotemporal hairline recession. Currently, there are no evidence-based studies to guide treatment for patients with FFA; thus, treatment options vary among clinicians. We report clinical findings and treatment outcomes of 36 patients with FFA, the largest cohort to date. Further, we report the first evidence-based study of the efficacy of hydroxychloroquine in FFA using a quantitative clinical score, the Lichen Planopilaris Activity Index (LPPAI). A retrospective case note review was performed of 36 adult patients with FFA. Data were collected on demographics and clinical findings. Treatment responses to hydroxychloroquine, doxycycline and mycophenolate mofetil were assessed using the LPPAI. Adverse events were monitored. Most patients in our cohort were female (97%), white (92%) and postmenopausal (83%). Apart from hairline recession, 75% also reported eyebrow loss. Scalp pruritus (67%) and perifollicular erythema (86%) were the most common presenting symptom and sign, respectively. A statistically significant reduction in signs and symptoms in subjects treated with hydroxychloroquine (P < 0·05) was found at both 6- and 12-month follow up. In FFA, hairline recession, scalp pruritus, perifollicular erythema and eyebrow loss are common at presentation. Despite the limitations of a retrospective review, our data reveal that hydroxychloroquine is significantly effective in reducing signs and symptoms of FFA after both 6 and 12 months of treatment. However, the lack of a significant reduction in signs and symptoms between 6 and 12 months indicates that the maximal benefits of hydroxychloroquine are evident within the first 6 months of use.