Lichen Planopilaris


Article Author:
Kenia Lepe


Article Editor:
Francisco Salazar


Editors In Chief:
David Wood
Andrew Wilt
Hajira Basit


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
6/4/2019 6:54:30 PM

Introduction

Lichen planopilaris is an inflammatory, primary cicatricial alopecia with several different patterns of hair loss. Like lichen planus, the etiology of lichen planopilaris is not known but presumably related to the cause of lichen planus.[1][2][3]

Etiology

Lichen planopilaris is the prototypical lymphocytic cicatricial alopecia according to the North American Hair Research Society (NAHRS). It is considered a follicular variant of lichen planus, based on clinical and histopathological findings. The most widely accepted theory states that it is a hair-specific autoimmune disorder in which activated T lymphocytes target follicular antigens.[4][5]

Epidemiology

The incidence of any of the cicatricial alopecias is not precisely known. It has been reported as the most frequent primary scarring alopecia, accounting for 43% in a 72 patient series. Frontal fibrosing alopecia and Graham-Little syndrome are considered variants of lichen planopilaris. It affects women more often than men, usually from 40 to 60 years of age. Up to 50% of patients may develop characteristic lichen planus lesions that affect the skin, mucous membranes or nails.

Pathophysiology

Cell-mediated immunity plays a major role in triggering the clinical expression of lichen planopilaris. It is proposed that this cell-mediated reaction is potentially initiated by the action of an endogenous or exogenous agent, such as drugs, virus, or contact sensitizers which bind to keratinocytes as well as follicular epithelium. Contact sensitizers such as metals including gold, mercury, and cobalt; could act as haptens and evoke an inflammatory reaction. Microorganisms that may play a role include hepatitis C virus, HIV, herpes simplex virus type 2, human papillomavirus and syphilis. Drugs probably involved include antimalarial agents, gold, beta-blockers, thiazides and angiotensin-converting enzyme inhibitors. After this initial trigger, keratinocyte and hair follicle may act as signal transducers, capable of converting these stimuli into producing cytokines and chemotactic factors for initiation and perpetuation of inflammation. Most infiltrated T lymphocytes are located around the bulge area. Lineage studies have proven that bulge area cells are multipotent and that their progeny generates the new lower anagen hair follicle. The failure of affected follicles to regenerate is thought to be due to the destruction of follicular stem cells located in the bulge area.[6][7][8]

Histopathology

A band-like mononuclear infiltrate obscuring the interface between the follicular epithelium, and the dermis is found. The epithelial-stromal junction may have prominent vacuoles and dyskeratosis with individually necrotic, polygonal basal keratinocytes. Inflammation affects the upper portion of the follicle most severely, but it may extend down the length of the follicle. Perifollicular fibrosis and chronic inflammation may be seen in later stages. Ultimately, follicles are replaced by columns of sclerotic collagen, called follicular scars. Such scars can be highlighted with elastic tissue stains. Grouped globular immunofluorescence, usually IgM, especially when found adjacent to the follicular epithelium is the characteristic pattern seen in lichen planopilaris, in contrast to chronic cutaneous lupus erythematosus where linear deposits of immunoreactants are typical.[9]

History and Physical

The clinical course of hair loss in lichen planopilaris may be insidious or fulminant and the pattern highly variable. Commonly, there are several scattered foci of partial hair loss with associated perifollicular erythema and scaling. A pattern of hair loss suggestive of central centrifugal alopecia or Brocq’s alopecia can also occur. The scalp lesions may be single or multiple, focal or diffuse, and occur anywhere on the scalp. Hair follicles around the margins of the bare areas show perifollicular erythema and perifollicular scale, in contrast to discoid lupus erythematosus where follicular plugging is in the center of the active bare patch. A pull test may yield anagen hair may yield anagen hair in cicatricial alopecia, which is a useful sign of active disease that requires treatment. Symptoms of itching, burning, pain, and tenderness are often severe. Cutaneous, nail and mucous membrane lichen planus may occur before, during or after the onset of scalp involvement. The most characteristic trichoscopic feature of active lichen planopilaris is perifollicular scaling. Trichoscopy of inactive end-stage lichen planopilaris reveals small, irregularly shaped, whitish areas lacking follicular openings, called “fibrotic white dots,” and white areas of conducted fibrosis.

Evaluation

The diagnosis is based on the clinicopathologic correlation; it cannot be made only by clinical signs and symptoms alone. A 4-mm-deep punch biopsy specimen should be submitted for horizontal sectioning and hematoxylin-eosin staining. The best biopsy site is an active, symptomatic hair-bearing area with perifollicular erythema and perifollicular scale, located at the margin of a bare patch, with positive anagen pull test.

Treatment / Management

Treatment aims to reduce hair loss, control the symptoms and stop the scarring process. Regrowth should not be expected since the complete elimination of inflammation is unlikely. As there are no consistent markers to measure the progress of the disorder, therapy is based on perceived severity and patient tolerance to treatment. Duration of treatment should be guided by clinical response and relapse rates. General measures include avoiding chemical or physical insults to the hair (coloring or perming). Contrary to many patients beliefs, the frequency of shampooing does not make a difference in the total number of hairs lost. Potent corticosteroids and topical tacrolimus are commonly used in all forms of primary cicatricial alopecia and frequently considered first-line treatments. Antimalarial drugs are commonly used to treat lichen planopilaris. Hydroxychloroquine 200 mg twice daily is generally used and is often considered first-line systemic therapy. Improvement is usually seen within 6 months. Minoxidil helps maximize hair growth of the remaining follicles. A new scoring system to assess response to treatment (lichen planopilaris activity index) has been proposed to document the course and response to treatment. It assigns numeric values to subjective and objective markers of the disease: symptoms (pruritus, pain, burning) and signs (erythema, perifollicular erythema, perifollicular scale), a measure of the activity (anagen pull test) and spreading of the condition. It intends to allow statistical comparison of pretreatment and posttreatment response.

Differential Diagnosis

The most important differential diagnosis may be seborrheic dermatitis since many patients have a long history of scalp scaling often diagnosed initially as seborrheic dermatitis. A sudden onset of patchy hair loss on the scalp may be diagnosed as alopecia areata, but when only partial hair loss, perifollicular erythema, and scaling are present within the patch, lichen planopilaris should be the main diagnostic suspect.

Prognosis

It is important to note that even if a primary scarring alopecia is controlled for a prolonged period, it may recur at any time.

Enhancing Healthcare Team Outcomes

Lichen planopilaris is best managed by a dermatologist. However, the patient may initially be seen by a general provider or nurse practitioner. It is important for these clinicians to refer patients with complex skin disorders to the respective specialist for treatment. Treatment aims to reduce hair loss, control the symptoms and stop the scarring process. Regrowth should not be expected since the complete elimination of inflammation is unlikely. As there are no consistent markers to measure the progress of the disorder, therapy is based on perceived severity and patient tolerance to treatment. Duration of treatment should be guided by clinical response and relapse rates. General measures include avoiding chemical or physical insults to the hair (coloring or perming). Some patients may benefit from hydroxychloroquine and minoxidil but a complete response is rare. Improvement, when it occurs, may take 6-9 months or even longer.

 

 


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Lichen Planopilaris - Questions

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Which of the following is a feature of lichen planopilaris helpful to distinguish it from discoid lupus erythematosus?



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Which of the following is considered first-line systemic therapy for lichen planopilaris?



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A 50-year-old woman presents with a 3-month history of progressive hair loss, associated with moderate pruritus and itching. Evaluation reveals 2 small patches with partial hair loss on the scalp, with associated perifollicular erythema and perifollicular scaling. The pull test on the margin of the patches is positive. Which of the following is the most likely diagnosis?



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Lichen planopilaris belongs to which category of primary cicatricial alopecias according to the North American Hair Research Society?



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Which is the most characteristic trichoscopic feature of active lichen planopilaris?



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A 49-year-old female presents the clinic for a follow-up examination. Three months earlier, a scalp biopsy had been performed, which showed a lymphocytic perifollicular band-like infiltrate along with reduced hair follicles. She states that itching and redness on her scalp have improved after 3-month treatment with hydroxychloroquine. On clinical examination, perifollicular scaling and erythema have diminished, partial hair regrowth is seen, although some hairless areas persist. Which of the following pathogenic mechanisms most likely explains the non-reversible nature of alopecia in this patient?



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A 45-year-old female presents to the clinic with a six-month history of hair loss. She states her scalp often itches and burns. On examination, two small alopecic patches are found on the parietal region of her scalp. Mild perifollicular erythema and perifollicular scale are found. Which of the following is the best initial therapy for this patient?



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Lichen Planopilaris - References

References

Ocampo-Garza J,Tosti A, Trichoscopy of Dark Scalp. Skin appendage disorders. 2018 Nov;     [PubMed]
Hu AC,Chapman LW,Mesinkovska NA, The efficacy and use of finasteride in women: a systematic review. International journal of dermatology. 2019 Jan 3;     [PubMed]
Lee B,Elston D, The Uses of Naltrexone in Dermatological Conditions. Journal of the American Academy of Dermatology. 2018 Dec 21;     [PubMed]
Babahosseini H,Tavakolpour S,Mahmoudi H,Balighi K,Teimourpour A,Ghodsi SZ,Abedini R,Ghandi N,Lajevardi V,Kiani A,Kamyab K,Mohammadi M,Daneshpazhooh M, Lichen planopilaris: Retrospective study on the characteristics and treatment of 291 patients. The Journal of dermatological treatment. 2018 Nov 9;     [PubMed]
Kelati A,Mernissi FZ, Central Frontoparietal Band-Like Alopecia in a 40-Year-Old Woman. Skin appendage disorders. 2018 Oct;     [PubMed]
Taguti P,Dutra H,Trüeb RM, Lichen Planopilaris Caused by Wig Attachment: A Case of Koebner Phenomenon in Frontal Fibrosing Alopecia. International journal of trichology. 2018 Jul-Aug;     [PubMed]
Doche I,Wilcox GL,Ericson M,Valente NS,Romiti R,McAdams BD,Hordinsky MK, Evidence for neurogenic inflammation in lichen planopilaris and frontal fibrosing alopecia pathogenic mechanism. Experimental dermatology. 2018 Nov 8;     [PubMed]
Imhof RL,Chaudhry HM,Larkin SC,Torgerson RR,Tolkachjov SN, Frontal Fibrosing Alopecia in Women: The Mayo Clinic Experience With 148 Patients, 1992-2016. Mayo Clinic proceedings. 2018 Nov;     [PubMed]
Nasiri S,Bidari Zerehpoosh F,Abdollahimajd F,Younespour S,Esmaili Azad M, A comparative immunohistochemical study of epidermal and dermal/perifollicular Langerhans cell concentration in discoid lupus erythematosus and lichen planopilaris: a cross-sectional study. Lupus. 2018 Dec;     [PubMed]

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