Esophageal lichen planus: Current knowledge, challenges and future perspectives

Abstract

Lichen planus (LP) is a frequent, chronic inflammatory disease involving the skin, mucous membranes and/or skin appendages. Esophageal involvement in lichen planus (ELP) is a clinically important albeit underdiagnosed inflammatory condition. This narrative review aims to give an overview of the current knowledge on ELP, its prevalence, pathogenesis, clinical manifestation, diagnostic criteria, and therapeutic options in order to provide support in clinical management. Studies on ELP were collected using PubMed/Medline. Relevant clinical and therapeutical characteristics from published patient cohorts including our own cohort were extracted and summarized. ELP mainly affects middle-aged women. The principal symptom is dysphagia. However, asymptomatic cases despite progressed macroscopic esophageal lesions may occur. The pathogenesis is unknown, however an immune-mediated mechanism is probable. Endoscopically, ELP is characterized by mucosal denudation and tearing, trachealization, and hyperkeratosis. Scarring esophageal stenosis may occur in chronic courses. Histologic findings include mucosal detachment, T-lymphocytic infiltrations, epithelial apoptosis (Civatte bodies), dyskeratosis, and hyperkeratosis. Direct immuno-fluorescence shows fibrinogen deposits along the basement membrane zone. To date, there is no established therapy. However, treatment with topical steroids induces symptomatic and histologic improvement in two thirds of ELP patients in general. More severe cases may require therapy with immunosuppressors. In symptomatic esophageal stenosis, endoscopic dilation may be necessary. ELP may be regarded as a precancerous condition as transition to squamous cell carcinoma has been documented in literature. ELP is an underdiagnosed yet clinically important differential diagnosis for patients with unclear dysphagia or esophagitis. Timely diagnosis and therapy might prevent potential sequelae such as esophageal stenosis or development of invasive squamous cell carcinoma. Further studies are needed to gain more knowledge about the pathogenesis and treatment options.

Keywords: Budesonide; Dysphagia; Esophagitis; Lichen planus; Precancerosis; T-lymphocytes.

Figure 1

Endoscopic findings in esophageal lichen planus. A: Trachealization; B: Trachealization and fragile mucosa; C: Hyperkeratosis; D: Hyperkeratosis and stenosis; E and F: Tearing and localized denudation of the mucosa; G-I: Tearing and spacious denudation of the mucosa. Endoscopic images were taken from our cohort of patients.

Figure 2

Histologic findings in esophageal lichen planus. A and B: Lichenoid lymphocytic infiltrate of the lamina propria spilling over to the partially detached squamous epithelium; B and C: Intraepithelial lymphocytosis associated with apoptotic squamous cells (Civatte bodies, arrows); D: Dense CD3+ T-cell rich inflammation of the lamina propria involving 2/3 of surface epithelium and muscularis; E: Presence of a CD4+T-cell subset in the infiltrate; F: Civatte body rimmed by CD3+ T-cells.

Figure 3

Esophageal epidermoid metaplasia in esophageal lichen planus. A-C: Atrophic squamous epithelium showing extensive detachment from the lamina propria, subtle hyperkeratosis (A, C) and mild intraepithelial CD3+ T-lymphocytosis (B) associated with scattered Civatte bodies (C, arrow); D, and E: Low-grade squamous orthokeratotic dysplasia in detached epithelium of ELP. Presence of basal-type cells in the lower half of the flat epithelium, note presence of scattered mitosis (E, star); F: An increased Ki67+ proliferation index.

Figure 4

Direct immunofluorescence. Fibrinogen deposits in the basal membrane as a characteristic feature of Lichen planus. Direct immunofluorescence image was taken from one of our patients.

Figure 5

Proposal for management of esophageal lichen planus. * As topical steroids (e.g. budesonide or fluticasone), swallowed spray, viscous solution, or orodispersable tablets might be adminstered. LP: Lichen planus; DIF: Direct immunofluorescence; ELP: Esophageal lichen planus.