Esophageal lichen planus: towards diagnosis of an underdiagnosed disease

Abstract

Background: Although lichen planus (LP) is a common skin disorder, the prevalence of esophageal involvement (ELP) and its clinical manifestations are poorly defined. We aimed to establish diagnostic criteria and characterize disease outcomes of ELP.

Methods: Clinical, endoscopic, histological, and immunofluorescence data from consecutive patients with known LP between 2013 and 2018 were analyzed. We established endoscopic (denudation and tearing of the mucosa, hyperkeratosis and trachealization) and histological criteria (mucosal detachment, T-lymphocytic infiltrate, intraepithelial apoptosis, dyskeratosis, and fibrinogen deposits along the basement membrane) to grade disease severity. Endoscopic findings were correlated with clinical symptoms. Response to medical therapy was monitored.

Results: Fifty-two consecutive patients (median age 59.5 years) were analyzed. According to our grading system, 16 patients were considered as severe and 18 as mild ELP. Dysphagia was the only symptom which differentiated patients with severe (14/16) or mild ELP (8/18) from patients without ELP (1/18). Concomitant oral and genital involvement of LP was associated with the presence of ELP, while oral involvement alone was not. Follow-up of 14/16 patients with severe EPL for at least one year revealed that most of these patients responded to topical corticosteroids (budesonide: n = 9/10 or fluticasone n = 2/2). Three budesonide patients experienced a resolution of symptomatic esophageal stenosis.

Conclusions: Esophageal involvement of LP is frequent, but may be asymptomatic. ELP can be diagnosed using the diagnostic criteria proposed here. Dysphagia and combined oral and genital manifestation are associated with ELP. Therapy with topical corticosteroids appears to be a prudent therapeutic approach for ELP.

Keywords:

• Lichen planus
• esophagus
• budenoside
• dysphagia
• esophagitis
• eosinophilic esophagitis
• skin disease
• autoimmunity
• T-cells

Acknowledgement

We thank Kaethe Thoma for excellent technical assistance in IF diagnostics and Gillian Marsden for language editing.

Author contributions

Wolfgang Kreisel, Johannes Steffen Kern, Kristin Technau-Hafsi and Franziska Schauer designed the study.

Endoscopies were performed by Wolfgang Kreisel, Henning Schwacha, Volker Brass, and Steffen Heeg.

Dermatological examinations were performed by Johannes Steffen Kern, Kristin Technau-Hafsi, and Franziska Schauer

Annette Schmitt-Graeff examined the histological sections and evaluated the immunohistochemistry of the esophagus. Kristin Technau-Hafsi and Johannes Steffen Kern evaluated histological sections of the skin.

Direct immunofluorescence was evaluated by Johannes Steffen Kern, and Franziska Schauer.

Carmen Monasterio, Franziska Schauer, Adhara Lazaro, Peter Deibert, Armin Kuellmer, Arthur Schmidt, Peter Hasselblatt, and all other authors participated in acquisition and analysis of the data.

Carmen Monasterio, Franziska Schauer, Adhara Lazaro, and Wolfgang Kreisel drafted the manuscript.

All authors had access to the study data and had reviewed and approved the final manuscript.

Disclosure statement

The authors have nothing to disclose. There was no financial support for the study.