Different Patterns of Lichen Planus in Three Members of One Family

Lichen planus

Background: Frontal fibrosing alopecia (FFA) is a primary lymphocytic scarring alopecia occurring mainly in postmenopausal women. A range of facial lesions have been described in FFA, such as lichen planus (LP) pigmentosus, red dots, facial papules, and perifollicular and diffuse erythema. These lesions can be the first sign of FFA. LP pigmentosus is a rare variant of LP. The first description of LP pigmentosus associated with FFA (in 2012) reported 22 cases of LP pigmentosus among 44 cases of FFA affecting South African patients. Methods: We reviewed 16 FFA patients with LP pigmentosus and the histopathological findings of the biopsy of LP pigmentosus in 9 patients. Results: Most patients had intermediate skin phototypes (III-IV; n = 10; 62%). The age at onset of LP pigmentosus ranged from 30 to 60 years. The most common histopathological findings were epidermal atrophy, basal cell degeneration, interfollicular inflammatory infiltrate and melanophages, and perifollicular changes. Other findings not previously described in LP pigmentosus were inflammation and interface changes on sweat duct epithelia (acrosyringium and superior dermal duct), and lichenoid perisebaceitis. Conclusions: Histology of our cases confirmed previous findings and showed a high incidence of perifollicular involvement with occasional changes affecting sebaceous and sweat glands.

Frontal Fibrosing Alopecia (FFA) is a progressive lymphocytic scarring alopecia characterized by a band-like area of frontal or frontotemporal hairline recession most commonly in postmenopausal women. Eyebrows, eyelashes, and sideburns as well as limb, axillary, moustache, beard, and pubic hair involvement can be present too. The dermatological literature is currently exploding in papers on FFA, therefore this chapter aims to summarize curious/novel trichoscopic-pathologic correlations. The pathogenesis of FFA is unclear but considered to be overlapping with lichen planopilaris (LPP) (see Lichen Planopilaris). Recent genome-wide association study showed that FFA is a genetically predisposed immuno-inflammatory disorder driven by HLA-B*07:02 allele on chromosome 6. There are three most common clinical patterns: (1) a linear uniform band of hair loss of the frontal hairline, (2) an irregular or zigzag pattern at and behind the frontal hairline, and (3) a ‘pseudo-fringe-sign pattern’ with hair loss behind the preserved frontal hairline. Several unusual variants have been identified including: (1) androgenetic alopecia-like pattern, (2) cockade-like pattern, and (3) ophiasis-like pattern (Figure 14.1). Other unusual variants include occipital FFA, patchy FFA and upsilon-like FFA. Figure 14.1 The 3 unusual clinical subtypes in FFA: (A) AGA-like pattern, (B) cockade, and (C) ophiasis pattern. https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_1.tif"/> Two curious associations: Lichen planus pigmentosus (LPPigm) is more prevalent in FFA patients with darker skin, can precede the diagnosis and presents as brown macules coalescing into patches on the face and upper trunk; (yellow) facial papules are skin-colored monotonous papules on the face, usually the temporal and malar area. Both features have been found more prevalent among premenopausal and Hispanic/Latino women (Figure 14.2). Figure 14.2 Two curious associations with FFA: (A) LPPigm (diffuse brown patches on face, note involvement of the upper eyelids) and (B) yellow facial papules. https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_2.tif"/> On trichoscopy the features are similar to LPP (see Chapter 13). Some additional or distinct features include: Loss of vellus hairs in the hairline (Figure 14.3A): while this is the rule, there can be exceptional/early cases of retained vellus hairs (Figure 14.3B) Figure 14.3 99Loss of vellus hairs in the frontal line in FFA (A) compared to normal hairline (B, ×20). (C) FFA with preserved vellus hairs but peripilar casts in the immediate vicinity behind (×10). https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_3a.tif"/> https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_3b.tif"/> 100Lack of tufts (compound hairs) compared to LPP (unpublished data; personal communication with Dr. Giselle Martins); the histologic correlation to this is that in FFA there are usually “eyes” and not “goggles” (see Figure 14.10) Peripilar casts are usually more subtle and absent in the sideburns (Figure 14.4) Figure 14.4 Preauricular areas/sideburns show lack of peripilar casts but transparent proximal emergences (×20). https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_4.tif"/> Vessel net (this corresponds to the superficial vascular plexus with its branching vessels visualized by trichoscopy due to the atrophy of the skin) (Figure 14.5) Figure 14.5 Vessel net in FFA (×40). https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_5.tif"/> Peripilar hypopigmented halo (Figure 14.6) Figure 14.6 Peripilar hypopigmented halo in FFA (×10). https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_6.tif"/> Eyebrow trichoscopy can show yellow dots, dystrophic hairs, gray dots and hairs growing in different directions (Figure 14.7). Trichochorrhexis nodosa can also be observed most likely due to aggressive topical application (personal observation) (Figure 14.8) Figure 14.7 (A) Eyebrow trichoscopy in FFA: yellow, gray dots and hair growing in distinct directions. (B) Dystrophic hairs: this patient has not plucked the eyebrows within last 6 months (×40). https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_7.tif"/> Figure 14.8 (A) Trichorrhexis nodosa of the eyebrows in FFA. The patient has been rubbing pimecrolimus cream into the affected eyebrows (B, ×20). https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_8.tif"/> LPPigm: folliculocentric blue-gray dots in circles, speckled dots, and rhomboid structures (Figure 14.9). In melasma the pigment is not folliculocentric Figure 14.9 (A) LPPigm on the forehead extending to the frontal hairline in FFA shows blue-gray dots in circles (×50) which is a clue to (B) periappendigeal involvement with accentuation of melanophages. https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9780429457609/a1a07c34-5530-453d-aca4-02e4ee680a4a/content/fig14_9.tif"/>

A 50-year-old woman of south Asian descent presented with a 4-year history of hair loss, itching and follicular prominence at her frontal hairline, in association with increasingly sparse eyebrows and loss of body hair. She was otherwise well and on no medication. Clinical examination revealed typical features of frontal fibrosing alopecia (FFA), with associated papules on the forehead. In addition, striking hyperpigmentation was evident over the forehead, face and neck. This was felt to be in keeping with FFA-associated dyspigmentation–lichen planus (LP) pigmentosus. Initial management was lymecycline 408 mg once daily, tacrolimus 0.1% ointment twice daily plus mometasone furoate 0.1% two times weekly to the neck, and pimecrolimus cream twice a day to the face, as well as a sun protection factor 50 sunscreen. Hydroxychloroquine was avoided initially due to the potential side-effect of altered skin pigmentation with this drug. While the hairline recession remained unchanged, the pigmentary changes worsened despite treatment and affected areas became increasingly itchy. Lymecycline was stopped and hydroxychloroquine 200 mg once daily was started. After 12 months, the cutaneous pigmentation continued to worsen and was extending onto the arms and torso. A trial of low-dose isotretinoin (20 mg once daily) was considered as this has been reported to be of benefit in LP pigmentosus. However, after 6 months, there was no improvement. A trial of mycophenolate mofetil 500 mg twice daily was then commenced. After 3 months of treatment, skin itching had settled and no new areas of hyperpigmentation were noted. After 9 months, there had been a significant improvement in skin pigmentation, which has been maintained. The extent of frontal hairline recession has remained stable. While FFA was initially described exclusively in postmenopausal White women, it has since been reported in Hispanic, African, Afro-Caribbean and Asian individuals, premenopausal women, and men. Dyspigmentation associated with FFA has largely been a feature observed in patients with darker skin types, and it is not clear whether this pigmentation is part of the spectrum of FFA in individuals with darker skin or whether LP pigmentosus is occurring coincidently with FFA in patients with darker skin types. Low-dose isotretinoin has been reported as being of benefit in LP pigmentosus but was ineffective in this case. Mycophenolate mofetil is also reported to be of benefit in the management of LP pigmentosus, and led to a significant improvement in both pigmentation and itch in this case. This case highlights the therapeutic benefit of mycophenolate mofetil in in LP pigmentosus associated with FFA.

Frontal fibrosing alopecia (FFA) is a distinctive lymphocytic scarring alopecia with rapid increase in prevalence. Most FFA series are retrospectively reported from Caucasians with only few from Asians. The objective of this study was to characterize the clinical, trichoscopic and histopathological findings as well as treatment outcomes. This was a retro-prospective cohort study of patients diagnosed with FFA from 1January 2010 to 1November 2019. All patients were asked to present for re-examination. Clinical, trichoscopic, histopathological and laboratory data were recorded. A questionnaire was used to investigate hair care, hairstyle and facial skin care compared with age-matched normal controls. Multivariate analysis was performed in order to clarify factors associated with severity. All 58 FFA patients were female, of whom 27.6% were premenopausal, 37.7% had a history of surgical menopause, 13.8% had thyroid diseases, 69% had eyebrow loss and 32.8% facial papules. On physical examination, 10.3% showed linear pattern, 46.6% diffuse pattern and 43.1% pseudo-fringe sign. Concomitant lichen planopilaris was found in 25.9%, lichen planus pigmentosus in 24.1% and female pattern hair loss in 48.3%. The most common trichoscopic characteristics in the frontal hairline were lack of follicular ostia (91.4%), perifollicular scales (79.3%) and perifollicular erythema (63.8%). Up to 90% of patients reported FFA as improved or stable after receiving antiandrogen (finasteride or dutasteride) or antimalarial with topical treatment. Multivariate analyses revealed that facial lentiginous macules and trichoscopic perifollicular erythema at the frontal area were FFA severity-associated factors. "Front puff" Thai hairstyle was associated with FFA, while sunscreens and other cosmetic products were not. In conclusion, diffuse and pseudo-fringe sign pattern are common in Asian FFA. The most common autoimmune systemic comorbidity is thyroid disease, while common concomitant dermatological diseases are female pattern hair loss, lichen planopilaris and lichen planus pigmentosus. Antiandrogens or antimalarial plus topical treatment are the most useful therapy.

Extensive facial scarring after ablative laser resurfacing in a patient with frontal fibrosing alopecia

Frontal fibrosing alopecia, just lichen planopilaris?

Topical JAK inhibitors in the lichen planus spectrum: a systematic review.

Frontal fibrosing alopecia (FFA), a form of lichen planopilaris (LPP), is primary cicatricial alopecia commonly affecting postmenopausal women. For the first time, we investigated the diagnosis of FFA and LPP in patients presenting with the chief complaint of facial papules and roughness. This cross-sectional was performed among 68 patients with facial papules. We described the epidemiology, comorbidities, clinical presentations, and the association between facial papules and LPP or FFA. All the patients were female with a mean age of 47.84 years. Scalp alopecia was observed in all the patients presenting with facial papules, of which 89.7% had FFA. Five patients were diagnosed with LPP without FFA. Most of the patients were premenopausal (73.5%), and 70.6% had grade I FFA. Concomitant cutaneous lichen planus involvement was observed more frequently than mucosal involvement. The most frequent comorbidities were hypothyroidism, dyslipidemia, and hypertension. History of alopecia areata was detected in 8.8% of the patients. Androgenetic alopecia (AGA) was present in 17 patients (25%). Facial papules are the silent and early signs of FFA and LPP. Paying attention to these early signs along with metabolic disturbances can help with the early diagnosis of the disease, especially among premenopausal women.

Introduction: Frontal fibrosing alopecia (FFA) is characterized by irreversible, symmetrical band-like hair loss in the frontotemporal region. Lichen planus pigmentosus (LPP) is a variant of lichen planus (LP) that presents with hyperpigmented macules and patches predominantly in sun-exposed areas. Nail LP is a subtype of LP that can be present alone or with other forms of LP. Case Report: We report a rare case of a 59-year-old woman presenting with symmetrical, gray-brown, hyperpigmented lesions on her neck and face, band-like alopecia in the frontotemporal region, severe onycholysis in two fingernails, and prominent longitudinal ridging in all fingernails. Clinical, dermoscopic, and histological findings established a diagnosis of FFA associated with LPP and nail LP was established. Discussion/Conclusion: In recent years, it has been established that FFA can be associated with LPP and it is thought to be a variant of lichen planopilaris. Nail involvement is rarely reported in FFA or LPP. To our knowledge, the presence of the three conditions in the same patient has not been previously reported. Although rare we would like to emphasize the importance of a careful examination of the nails in patients with FFA and/or LPP to prevent irreversible nail changes.

Frontal fibrosing alopecia in men: A multicenter study of 39 patients

Frontal fibrosing alopecia (FFA) is a primary cicatricial alopecia first described by Kossard in 1994. It is considered a clinical variant of lichen planopilaris (LPP). In the last two decades, there have been an explosion of cases worldwide. While predominately seen in Caucasian, post-menopausal women, it has been reported in various ethnicities including African-Americans, Hispanics, Asians, and Indians as well as in pre-menopausal women and men.1-5 Characterized by progressive, scarring frontotemporal hair loss with perifollicular erythema, follicular keratinization, and reduced follicular orifices, the most common finding is bandlike recession of the scalp hairline.1-5 Accompanying features include eyebrow thinning, eyelash loss, body hair loss, facial papules, lonely hairs, and occipital alopecia.1-5 While typically asymptomatic, pruritus and trichodynia can occur. Eyebrow thinning often presents prior to scalp hairline regression and has been seen with milder hairline regression compared to individuals who do not experience eyebrow thinning first.2-4 Other dermatoses seen in patients with FFA include lichen planus on regions other than the scalp and lichen planus pigmentosus in non-Cacuasians. This article is protected by copyright. All rights reserved.

Introduction: Frontal fibrosing alopecia (FFA) is a lymphocytic primary cicatricial alopecia typically involving the frontotemporal hairline. It may be associated with the presence of facial papules (FP) that clinically appear as noninflammatory, monomorphic, white-yellowish papules. Lichen planus pigmentosus (LPPigm) is characterized by the presence of asymptomatic grayish pigmented macules, predominantly in sun-exposed and flexural areas. Case Report: A 58-year-old, Caucasian, phototype III woman presented with a symmetrical, band-like, frontotemporal alopecia with regression of the hairline; bilateral eyebrow loss; diffuse, symmetrical hyperpigmentation of the face; and some asymptomatic, flesh-colored, monomorphic papules on the chin. Based on clinical, dermoscopic, and histological findings, the diagnosis of FFA associated with FP and LPPigm was established. Discussion/Conclusion: The peculiarity of our report is represented by the triple association of FFA, FP, and LPPigm in a Caucasian skin type III woman, as it has been rarely reported. Clinicians should be aware of this association also in subjects with phototype ≤III, as its recognition may be useful for diagnostic and prognostic purposes: the observation of LPPigm of the face may suggest to check for early FFA, and in case of FFA associated with FP, a poorer FFA prognosis may likely be expected.

Lichen planopilaris (LPP) is the most common primary cicatricial alopecia defined by the follicular form of lichen planus.1 Histopathologic examination is considered the gold standard diagnostic method. However, it may not always be diagnostically reliable.2 When there is histological ambiguity, trichoscopy is a highly effective tool for diagnosis and monitoring treatment effectiveness.1 Frequently reported trichoscopic findings of LPP include the absence of follicular openings, perifollicular scaling, milky-red areas, and blue-gray dots in a target pattern. Perifollicular scaling (casts) is considered the most characteristic trichoscopic feature of LPP and frontal fibrosing alopecia (FFA).3 It varied between 38% and 100% (mean: 85%) of patients with LPP and 42%–90% (mean: 84%) of patients with FFA.1 Peripilar casts represent hyperkeratosis and lamellar fibrosis resulting from perifollicular lichenoid inflammation, hence, considered ideal sites for biopsy.1, 4 Although tubular type of hair casts was considered specific for LPP they were also observed in discoid lupus erythematosus (DLE) and folliculitis decalvance (FD). Contrary to LPP and FFA perifollicular scaling in FD is characterized by a yellowish color.3 However, diagnosis may be challenging in some cases. Considering perifollicular scaling as a frequent and specific dermoscopic feature of active LPP, recognition of its dermoscopic details might help improve the accuracy of clinical diagnosis of LPP and FFA from other diseases causing cicatricial-alopecia. Concerning patterns of tubular hair cast, if combined with minimum or absence of interfollicular scales has been described as a specific dermoscopic feature of LPP and FFA by Mathar et al.4 Herein we describe a new dermoscopic pattern of tubular scaling that we detected exclusively in patients with active LPP and FFA lesions. “Double ring sign” consists of two white-silvery rings of scaling around hair follicle and is identified on dry dermoscopy. The inner ring migrates along the hair shaft and forms a tubular structure that was previously described in the literature.3 The outer ring is a little far from the hair shaft and more delicate than the inner ring (Figure 1A,B). In our experience double ring sign is a helpful dermoscopic finding for differentiating LPP and FFA from other cicatricial alopecia. We evaluated the available dermoscopic images of 102 pathologically confirmed cicatricial-alopecia patients with clinically active disease. These include 45 cases of LPP, 36 cases of FFA, 13 cases of DLE, and 8 cases of FD. Double ring sign were observed only in 24 (53.3%) and 13 (36.1%) patients with LPP and FFA, respectively but not in DLE and FD. Among patients with LPP and FFA, those with severe perifollicular scaling (p < 0.001) and positive anagen pull test (p < 0.05) were more likely to have double ring sign (Tables 1 and 2). To correlate dermoscopic-pathologic features a 4-mm punch-biopsy specimen from the scalp area presenting double ring sign was obtained in three patients after informed consent. The main histopathological findings include lichenoid infiltrates involving infundibulum and isthmus causing reactive infundibular hyperplasia with compact hyperkeratosis extending outward from the follicles (Figure 1C). Regarding presence of white-silvery scaling in dermoscopy corresponding to parakeratosis in histopathology,5 concentric rings of white scaling in LPP have a parakeratotic nature which is created reactive to perifollicular inflammatory infiltrate. In other words, there are alternating circumferential parakeratosis and hyperkeratosis around the hair shaft (Figure 1C,D). This indicates that a control mechanism operates to a variable degree in LPP lesions leading to decreased perifollicular infiltration. Therefore, alternation of parakeratosis and orthokeratosis often occurs in lesions that have not been treated. When better understood, it is suggested this mechanism might be utilized in treating or preventing disease. Probably an intermittent course is an integral part of autoimmune diseases. For instance, alopecia areata presents with Pohl-Pinkus constrictions.6 We believe along with LPP, in other cicatricial alopecia like DLE or folliculitis decalvance, the same intermittent inflammation may occur. However, we suggest the simultaneous affection of epidermis or interfollicular spaces in such cases mask the dermoscopic feature of hypothetical perifollicular inflammation fluctuation if existing. In conclusion, double ring sign is a new specific dermoscopic feature of LPP and FFA observed in approximately one-third to half of active lesions. Rings of white-silvery scaling seem to be reactive to “waves” of disease activity related to perifollicular inflammation. It could be easily identified on dry dermoscopy and can benefit clinicians to differentiate LPP and FFA from other causes of cicatricial alopecia. This observation should be validated in future prospective works with a greater sample size. Mina saber involved in research design, project supervision, manuscript writing, and editing. Farahnaz Fatemi Naeini involved in patient recruitment, data collection, and manuscript writing. None. The authors have no conflict of interest to declare. This study has been approved by the ethics committee of the Isfahan University of Medical Sciences. Informed consent for the publication of medical images was obtained from the patients.

The timing and distribution of nonscalp hair loss in patients with lichen planopilaris and frontal fibrosing alopecia: A survey-based study