Esophageal Lichen Planus: a Narrative Review of the Literature.

Lichen planus is a chronic inflammatory immune disorder that most commonly affects the skin and mucous membranes. Esophageal lichen planus (ELP) is a frequently misdiagnosed and poorly understood form of lichen planus that can be asymptomatic or present with dysphagia and odynophagia caused by the formation of erosions and strictures in the esophagus. These strictures often reduce a patient's quality of life and may lead to emaciation in more severe cases. We present the case of an 89-year-old woman with a history of cutaneous lichen planus (CLP) and mucosal lichen planus that were successfully managed with topical corticosteroids and oral cyclosporine rinses who presented with an esophageal stricture and erosions that were treated unsuccessfully with surgery. Our patient's condition continued to worsen until she presented in an emaciated state and was treated with tofacitinib, which resulted in complete resolution of oral lichen planus (OLP), ELP, and genital lichen planus.

INTRODUCTION: Lichen planus is an inflammatory disorder of the mucocutaneous tissues. Esophageal involvement is rare and is often asymptomatic early in disease course. Therefore, delay in diagnosis is common and may predispose patients to both failed symptom treatment and development of oral squamous cell carcinoma. Esophageal lichen planus (ELP) is a rare and underrecognized cause of dysphagia that has been reported almost exclusively in middle-aged to elderly women. We present a male patient with ELP and the uncommon endoscopic finding of esophageal pseudodiverticulosis and histology notable for Civatte bodies. CASE DESCRIPTION/METHODS: 36-year-old male presents with 4 years of dysphagia to solids that worsened following his fourth esophageal balloon dilatation for esophageal stenosis. He endorsed a pruritic rash on his torso, localized to his hands, arms, and abdomen (Figure A). EGD revealed multiple pseudodiverticula and a benign appearing stricture in the proximal esophagus at 15–18 cm from the incisors (Figure B). Random esophageal biopsies demonstrated increased intraepithelial lymphocytes with scattered Civatte bodies and rare eosinophils (Figure C). Patient’s hospital course was complicated by concurrent diarrhea; stool culture positive for Campylobacter and Clostridium difficile. Therefore, patient was started on topical steroids and oral antihistamine instead of the standard treatment of systemic steroids. DISCUSSION: ELP is a rare condition that often occurs in conjunction with other manifestations of lichen planus. Yet, ELP continues to be under-recognized due to misinterpretation of the endoscopic features. Predominant endoscopic finding of ELP include stricturing of the proximal esophagus as seen in our patient. The most suggestive evidence of ELP is the presence of atrophic epithelial changes, lymphohistiocytic inflammatory infiltrate, and scattered, degenerated and dyskeratotic cells also known as Civatte bodies. Mucosal changes from ELP varies, but in our patient, we found an uncommon endoscopic finding of esophageal pseudodiverticulosis. Based on previous case series and case reports, easy sloughing of the esophageal mucosa seems to be another characteristic sign of ELP. In conclusion, ELP is a rare condition that is commonly missed; the presence of esophageal pseudodiverticulosis may hint at ELP. It is critical to consider ELP in patients with dysphagia and dermatologic manifestations of lichen planus, as delayed diagnosis of ELP may result in oral squamous cell carcinoma.Figure 1.: Pruritic rash on torso, hands, arms, and abdomen with oral lesion on physical examination.Figure 2.: Image 1 & 2: EGD revealed multiple pseudodiverticula in the esophagus marked by arrows Image 3:Benign appearing stricture in the proximal esophagus at 15-18cm from the incisors.Figure 3.: Esophageal biopsy concerning for increased intraepithelial lymphocytes with scattered Civatte bodies and rare eosinophils. Arrows marking Civatte bodies.

Lichen planus

HomeRadioGraphicsVol. 43, No. 4 PreviousNext Cases from the Cooky JarFree AccessLichen PlanusEdward McDermott , Nadia Solomon, Annelise M. Silva, Parisa KhoshpouriEdward McDermott , Nadia Solomon, Annelise M. Silva, Parisa KhoshpouriAuthor AffiliationsFrom the Department of Radiology, Mater Misericordiae University Hospital, Eccles Street, Dublin D07 R2WY, Ireland (E.M.); Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, Conn (N.S.); Department of Education, Wright State University Boonshoft School of Medicine, Dayton, Ohio (A.M.S.); and Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Medical Institution, Baltimore, Md (P.K.).Address correspondence to E.M. (email: [email protected], @edwardmcd).Edward McDermott Nadia SolomonAnnelise M. SilvaParisa KhoshpouriPublished Online:Mar 16 2023https://doi.org/10.1148/rg.220200MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Lichen planus is a chronic condition that predominantly affects middle-aged and older women and represents an autoimmune T-cell–mediated inflammatory disorder (Fig 1) that affects the skin, oral and genital mucosa, esophagus, hair, and nails (1). Cutaneous involvement manifests with flat violaceous papules, and mucosal manifestations typically manifest with white lacelike lesions, papules, erythematous lesions, and erosions (Fig 2) (2). Esophageal lichen planus has been reported in more than 50% of patients with mucocutaneous lichen planus and is underrecognized and underreported (2).Figure 1. Esophageal lichen planus. There are limited studies of the pathogenesis of esophageal lichen planus, but it appears to be mediated by cytotoxic CD8-positive T cells. Biopsy of affected esophageal tissue shows keratinization with inflammatory cell infiltration of the epithelium with activated T lymphocytes (6).Figure 1.Download as PowerPointOpen in Image Viewer Figure 2. Mucosal manifestations of lichen planus in a 73-year-old woman who presented with solid food dysphagia. (A) Photograph shows white plaquelike lesions on the tongue. (B) Endoscopic image demonstrates erythema and white papules (arrows).Figure 2.Download as PowerPointOpen in Image Viewer Barium esophagography is the most relevant imaging study to evaluate esophageal lichen planus. Imaging features include a smooth small-caliber esophagus with cervical esophageal webs, strictures, and pseudodiverticula (Fig 3) (3,4). Strictures most commonly occur in the proximal esophagus, and their presence correlates with a longer duration of disease (5). Squamous cell carcinoma of the esophagus has been rarely reported in patients with esophageal lichen planus; for this reason, early diagnosis is key.Figure 3. Esophageal lichen planus in a 73-year-old woman (same patient as in Fig 2). Barium esophograms show smooth long stricturing of the entire course of the esophagus with tapered margins (white arrow in A) and esophageal webs (black arrows in A). There are focal weblike strictures in an overall small-caliber lumen of the cervical esophagus (black arrow in B). Overall thin smooth luminal narrowing of the entire course of the esophagus is seen in C.Figure 3.Download as PowerPointOpen in Image Viewer Patients with esophageal lichen planus often present with dysphagia related to the strictures, with reflux-type symptoms, and weight loss. Strictures can be treated with balloon dilation, although this often requires regular repeat treatments (5). Endoscopic findings include superficial white papules, pseudomembranes, webs, and strictures.Disclosures of conflicts of interest.—All authors have disclosed no relevant relationships.AcknowledgmentWe thank Ciléin Kearns, MD, MBChB, for feedback and advice on the medical illustration in Figure 1.All authors have disclosed no relevant relationships.

The evaluation of cutaneous, genital, scalp, nail, esophageal, and ocular involvement in patients with oral lichen planus

Case Presentation: A 71-year-old white female with a past medical history of gastroesophageal reflux disease presented with intermittent dysphagia to solid food and odynophagia that had been progressing for the past few months. She also reported unintentional weight loss. Patient has history of erosive lichen planus that was causing ulcers in her oropharynx three years ago and it was treated with topical steroids. All her initial laboratory tests were normal, this includes CBC, BAMP, LFTs. Barium sallow was performed and showed narrowing in the proximal one-third of the esophagus. Subsequent upper endoscopy revealed ulcerated circumferential stricture in the proximal esophagus just 2 cm below the cricopharyngeal muscle. Upper endoscopy also showed moderate mid and distal esophageal strictures (Figure. 1). Esophageal biopsies were consistent with acute erosive esophagitis with lymphocytic predominance and with minimal eosinophils. Based on the presence of oral lichen planus, our patient's endoscopic apperance and the biopsy findings, the patient was diagnosed with esophageal lichen planus. She had undergone multiple esophageal dilatations since the initial presentation. She had also received a course of systemic steroids. On follow-up, the patient was clinically stable and had denied worsening of her symptoms.Figure 1Discussion: Lichen planus is a common idiopathic disorder involving skin, nail, and mucosal membranes. It is characterized by immune response attacking an antigen in the basal cells of squamous epithelium. Esophageal lichen planus is a relatively rare condition and the prevalence is unknown. The diagnosis can be challenging due to the subtle clinical findings and lack of characteristic histologic features. Esophageal lichen planus usually affects the upper and mid esophagus; in addition, it often spares the gastroesophageal junction when compared to reflux esophagitis. The history of oral lichen planus, endoscopic images and site of esophageal involvement are very helpful in differentiating between the latter two entities. Esophageal lichen planus has a tendency to be chronic disease with the potential risk of malignancy. Topical and oral steroids are the mainstay of treatment but to date there are no wellestablished guidelines. Conclusions: Oropharyngeal lichen planus is a chronic disease that can lead to recurrent esophageal stricture. Endoscopic findings and pathology may not be specific; however, clinicians should be aware of this entity and appropriate referrals should be made as patients might need repeated interventions to have a relief in their symptoms.

Background: Lichen planus (LP) is a common inflammatory disease affecting skin, mucous membranes, hairs, and nails, with an unpredictable course involving remissions and relapses. LP is a Type-I-Inflammation disease involving IFN-γ and IL-17 as key inflammatory mediators. Materials and Methods: We searched PubMed/MEDLINE and Google Scholar search engines for studies on the esophageal manifestation of lichen planus over an unlimited time frame. Articles were searched with combinations of Medical Subject Heading (MeSH) terms. Given the limited number of publications, no exclusion criteria were applied. Results: Esophageal lichen planus (ELP) is an underreported manifestation of LP that primarily affects middle-aged women. Its prevalence among LP patients remains to be defined. Though potentially clinically silent, ELP can significantly impact patient wellbeing and serve as a precursor to esophageal squamous cell carcinoma. While dysphagia is the primary symptom, the condition may also remain subclinical. The endoscopic hallmarks of ELP are mucosal denudation and tearing, trachealization, and hyperkeratosis. Chronic disease progression may lead to scarring esophageal stenosis. Histologically, ELP shows mucosal detachment, T-lymphocytic infiltrations, epithelial cell apoptosis (Civatte bodies), dyskeratosis, and hyperkeratosis. Fibrinogen deposits along the basement membrane zone distinguish ELP from various immunological esophageal diseases. There is currently no standardized therapy available. Topical steroids lead to symptomatic and histologic improvements in two-thirds of patients. Severe or refractory cases require immunosuppressive therapy, whereas JAK-inhibitors represent a promising emerging option. Endoscopic dilation helps symptomatic stenosis. Considering ELP’s precancerous potential, timely diagnosis and treatment are crucial in preventing complications, such as stenosis or invasive esophageal squamous cell carcinoma. Conclusions: ELP is an underdiagnosed and underreported manifestation of LP. While it may remain clinically silent, it can nevertheless significantly affect patients’ wellbeing and life expectancy. This narrative review aims to initiate multidisciplinary cooperation among gastroenterologists, dermatologists, oral health professionals, and histopathologists to support clinical diagnosis and management.

Esophageal lichen planus is a rare condition. Its risk of malignant transformation is unknown. We report a series of eight patients with esophageal lichen planus referred to our unit between 1990 and 2005. Clinical, endoscopic, radiological and histological data of these patients were retrospectively reviewed. Seven patients were women. All patients had oral lichen planus. Endoscopic lesions were located in the upper third of the esophagus in seven patients and in the mid third in two patients. Five patients had esophageal stricture. Seven patients had peeling, friable esophageal mucosa. Histological examination of esophageal biopsies found characteristic features of lichen planus in two patients and nonspecific changes in five patients. All patients received corticosteroids. Patients with stricture underwent esophageal dilation. Esophageal perforation after dilation occurred in one patient. Corticosteroids improved dysphagia in all patients; steroid dependence occurred in two patients with stricture. One patient had an esophageal verrucous carcinoma, which was treated with radiotherapy and chemotherapy. Upper endoscopy should be performed in patients with mucosal lichen planus presenting with dysphagia to assess esophageal involvement. Esophageal strictures are frequent and require dilation. Corticosteroids are the first-line treatment, but steroid dependence may occur. Cancer can arise on esophageal lichen planus and justifies endoscopic follow-up.

Objective To explore and report the clinical oral lichen planus (OLP) cases combining with esophageal lichen planus (ELP) , including prevalence, clinical manifestations, diagnosis, treatment and prognosis. Methods Two hundred and thirty-six OLP patients with chief-compliances and symptomes such as chest discomfort, chest burning, dysphagia, poor food intake et al. were included in this study. Esophagogastroscopy and biosy were suggested and applied to find weather combining with esophageal lichen planus (ELP) after written informed consent were obtained. Standard therapy of OLP and case follow-up were applied. Results Eighteen OLP patients with combined ELP were found (7.6%, 18/236) . Dysplasia were detected in 2 cases 11.1% (2/18) and in 1 case, 5.5% (1/18) , esophageal squamous cell carcinoma was detected while the three OLP was not malignant-transformed. Conclusions Lichen planus is general condition and there is a correlation between ELP and OLP. Thus, oral medicine doctors should pay attention to the other site lichen planus lesion to detect early and hiding malignant-transformed, avoided misdiagnosis and missed diagnosis. Key words: Oral lichen planus; Esophageal lichen planus

An involvement of the esophagus in patients with lichen planus was described for the first time in 1982. Ever since, it has been seen as a rarity. However, studies over the last 10 years have shown a higher prevalence than expected. It may even be supposed that esophageal lichen planus (ELP) is more common than eosinophilic esophagitis. ELP mostly affects middleaged women. The principal symptom is dysphagia. Endoscopically, ELP is characterized by denudation and tearing of the mucosa, trachealization and hyperkeratosis and esophageal stenosis may occur in patients with long courses of the disease. Histologic findings including mucosal detachment, T-lymphocytic infiltrate, intraepithelial apoptosis (civatte bodies) and dyskeratosis are crucial. Direct immunofluorescence shows fibrinogen deposits along the basement membrane zone. So far, there is no well-established therapy but a treatment with topic steroids is effective in 2/3 of the patients. Common therapy of lichen planus of the skin seems to be ineffective for treatment of ELP. Symptomatic esophageal stenosis should be endoscopically dilated. ELP joins the group of "new" immunologic diseases of the esophagus.

Introduction: Esophageal lichen planus (ELP) is an unusual and under-recognized cause of dysphagia. Timely diagnosis is crucial for effective treatment as ELP has been associated with increased risk for development of squamous cell carcinoma. Case Description/Methods: A 72-year-old man presented with esophageal dysphagia to solids. Past medical history included oral lichen planus (LP) and osteoporosis with use of alendronate. He underwent endoscopy (EGD) which showed a proximal esophageal stricture for which balloon dilation ranging from 8 to 15 mm was done. Biopsy of the stricture showed esophageal mucosa with increased intraepithelial eosinophils (Figure). The working diagnosis was peptic stricture with possible eosinophilic esophagitis (EoE). He was started on pantoprazole 40 mg twice daily. He underwent 2 more EGDs with dilation for recurrent stricture and on the 2nd EGD, biopsy of the stricture showed numerous intraepithelial lymphocytes and focal apoptotic squamous cells (Civatte bodies) compatible with ELP. Budesonide slurry 1 mg twice daily was instituted and an EGD 6 months later showed near resolution of the stricture. The patient no longer requires dilations or antiacid therapy and is asymptomatic currently. He had Candida esophagitis 1 month after initiation of budesonide which was treated with fluconazole. Discussion: ELP is prevalent in patients with mucocutaneous forms of LP, most often oral LP. It is rarely seen in males with a case series by Franco et al describing the female to male ratio as 5:1. The endoscopic differential diagnosis for ELP should distinguish findings of esophagitis and stricture secondary to EoE, reflux esophagitis or esophageal Candidiasis. A low threshold should be kept for suspecting ELP in a patient with dysphagia and mucocutaneous LP. If histology is inconclusive but a high index of suspicion remains, particularly in patients with mucosal LP, repeating biopsies may be warranted for diagnosis as is highlighted in this case. A co-relation between longer duration of disease with severity of inflammation has been proposed with case series reporting strictures. Treatment includes serial dilations, topical and/or systemic steroids. Recurrence has been described despite topical steroid therapy with requirement of additional dilations. Successful use of immunomodulators such as cyclosporine, mycophenolate has been described. There is paucity of data regarding surveillance and optimal treatment for ELP, and thus further studies are needed for better understanding of this disease.Figure 1.: Esophageal biopsy showing esophageal squamous mucosa with basal cell hyperplasia, intraepithelial lymphocytes, and 2 apoptotic keratinocytes (Civatte or colloid bodies). Findings are compatible with esophageal lichen planus.

Lichen planus (LP) is a common disease of unknown etiology. Rare mucosal involvements like esophageal LP have been reported increasingly. Infectious agents including H. pylori and other autoantigens have been investigated in etiology and association with certain gastrointestinal pathologies have been well documented. The aim of this study is to investigate the upper gastrointestinal tract involvement in LP and to evaluate the possible etiologic role of H. pylori. 49 LP patients and 35 volunteers (without LP) with gastrointestinal symptoms were included in the study as the control group. LP group was divided into subgroups regarding gastrointestinal symptoms. Upper videoendoscopy was performed in both groups and biopsies were taken from suspicious areas for LP, gastrointestinal diseases, H. pylori and examined histopathologically. SPSS 13 was used for the analysis. Groups/subgroups were compared via xi-square test, Mann-Whitney U test, and t-test. Gastrointestinal symptoms were recorded in 71% of LP group; none of LP patients presented typical esophageal LP. Gastrointestinal diseases were more frequent in LP group than controls, endoscopically. Chronic gastritis (91.8%) was the leading diagnosis in LP patients. Superficial gastritis was significantly higher (13.3%) in LP patients than controls (p = 0.04). LP was not diagnosed in any of the esophageal mucosa biopsies whereas lymphoid follicles were observed significantly higher in control group (p < 0.01) histopathologically. H. pylori positivity was found higher in LP group (81.6%) though statistically insignificant. We believe upper endoscopy should be performed to investigate esophageal LP and gastrointestinal pathologies especially when LP patient is symptomatic. Although we didn't detect esophageal LP, our study had the limitation of taking biopsies from pathological sites only. Since histopathological examination of normal appearing esophagus may help in diagnosing occult LP, and prevent eventual complications, it may be further evaluated in larger study groups. A new technique, magnification chromoendoscopy, may be useful in detecting esophageal involvement. We believe the possible role of H. pylori in LP is yet to be determined also.

Lichen planus (LP) is an inflammatory condition that affects skin, hair follicles, nails and mucous membranes with esophageal involvement being an underrecognized manifestation. In this prospective cohort study (2020-2023), we screened 562 patients for symptomatic and clinically relevant esophageal lichen planus (ELP), using dysphagia as the primary screening criterion. The study included patients from the Department of Dermatology with either newly diagnosed or existing LP who reported esophageal symptoms, as well as patients referred from the Department of Gastroenterology who underwent endoscopy for unexplained esophageal symptoms potentially associated with ELP. The diagnosis of ELP was based on endoscopic, histopathological, and immunofluorescence findings. Through this approach, 77 patients with dysphagia and potential ELP were identified, of whom 21 were diagnosed with ELP. A more detailed evaluation of their symptoms revealed that ELP patients exhibited significantly higher rates of esophageal dysphagia, food bolus obstruction, and retrosternal pain compared to LP patients without esophageal involvement. Two ELP patients were diagnosed with esophageal squamous cell carcinoma. Multilocular LP manifestation emerged as a strong indicator of esophageal involvement. This study underscores the importance of recognizing ELP, recommending comprehensive endoscopic evaluation and dermatological assessments when esophageal symptoms are present.

Lichen planus (LP) is a classic skin disease that can involve the skin, hair, and nails, as well as the oral and genital mucosa. Histopathology is characterized by a T-lymphocytic, lichenoid, and interface dermatitis. Multiple case reports and small case series have shown that LP can involve the esophagus. However, the diagnostic criteria, incidence, and best treatment options remain uncertain. This study aimed to refine the diagnostic criteria, estimate prevalence, and present an outlook on treatment options to prevent long-term sequelae. Thirty-two consecutive patients with LP of the skin, hair, nails, oral mucosa, and/or genital mucosa underwent a comprehensive clinicopathologic assessment. Esophagogastroduodenoscopy was performed, and biopsies were evaluated histologically, immunohistochemically, and by direct immunofluorescence. Patients diagnosed with esophageal lichen planus (ELP) were followed up prospectively where possible. In total, 20 of 32 patients had ELP. Ten of these 20 patients were classified as having proven ELP, with clear-cut endoscopically visible lesions; the other 10 were classified as having probable ELP. Eight of 10 patients with proven ELP were started on new or additional therapy because of esophageal findings. Treatment with a topical budesonide formulation or systemic corticosteroids was successful in most patients with proven ELP and reversed functional esophageal stenosis. ELP can be found in more than 50% of patients with proven mucocutaneous LP when clinical and pathologic findings are correlated carefully. Topical or systemic corticosteroids are the first-line therapy for ELP. Timely medical therapy seems to prevent scarring stenosis of the esophagus.

Involvement of the esophagus by lichen planus is a rarely reported condition. The histologic features of esophageal lichen planus, which may differ from those of cutaneous disease, have only rarely been illustrated. We describe a 58-year-old woman with skin and oral lichen planus who presented with dysphagia and an esophageal stricture that were ultimately diagnosed as esophageal lichen planus. Multiple esophageal biopsies demonstrated a lichenoid, T cell-rich lymphocytic infiltrate, along with degeneration of the basal epithelium and Civatte bodies. Correct diagnosis of esophageal lichen planus is critical because of its prognostic and therapeutic distinction from other more common causes of esophagitis and stricture formation.