A 67-year-old woman with a long-standing history of recurrent dysphagia and esophageal strictures failed to respond to aggressive antireflux management. She required multiple dilations for symptomatic strictures that were discovered throughout the esophagus. Intralesional, topical, and systemic glucocorticoid therapies were utilized without resolution in symptoms. Several years after initial presentation, histopathology ultimately demonstrated lichenoid features and a diagnosis of esophageal lichen planus (ELP) was confirmed. However, as her symptoms had already become significantly disabling with severe strictures that carried an increased risk of endoscopic complications with dilation, she ultimately decided to undergo an esophagectomy for definitive treatment. Moreover, ELP may often go unrecognized for several years. Clinicians should consider ELP in the differential for dysphagia in middle- to elderly-aged women with or without a known history of lichen planus (LP) especially for those with findings of multiple or proximal strictures.
Lichen planus (LP), an inflammatory disorder with an unknown etiology, commonly manifests in the oral mucosa and skin [
A 67-year-old woman presented with a long-standing history of dysphagia and recurrent strictures. She did not have typical heartburn or regurgitation symptoms consistent with reflux disease. Her medical history included psoriasis and hypothyroidism. Exam findings of oral lesions or skin findings consistent with LP were not identified.
Laboratory investigations were remarkable for a positive antinuclear antibody with titres of 1 : 320 in a homogenous pattern with other autoimmune markers being nonreactive. Hepatitis C antibody was also nonreactive. Histopathology specimens predominantly showed nonspecific findings of acute and chronic inflammation suggestive of reflux disease.
She underwent multiple esophagogastroduodenoscopies (EGD) with findings of mucosal friability, webs, and strictures (Figures
Stricture in the lower third of esophagus.
Stricture in the middle third of esophagus.
Proximal stricture treated with dilatation.
Initial treatment consisted of twice daily proton pump inhibitor (PPI) for initially suspected reflux esophagitis, which offered no improvement in symptoms. A trial of inhaled swallowed fluticasone propionate 440 mcg twice daily for at one point suspected eosinophilic esophagitis provided no relief. Endoscopic bougie and balloon dilations provided transient improvement but this became less sustained over time. The primary modality of treatment that offered prolonged benefit was recurrent dilations with triamcinolone acetonide injections (10 mg/mL concentration) to strictures though the time interval of relief ultimately decreased as well. A trial of systemic glucocorticoids (40 mg/daily with a tapered course over 10 weeks) was also given for a suspected autoimmune etiology and did not provide any relief.
Seven years after her initial presentation, deep esophageal biopsy specimens were obtained showing severe acute and chronic esophagitis with a lichenoid-like pattern of chronic inflammation with notable features of lymphocytic infiltrate involving the basal layer of the epithelium and scattered apoptotic keratinocytes (Civatte bodies) (Figures
Esophageal squamous epithelium with H&E staining at 100x magnification. Findings showing lymphocytic infiltrate in the basal layer and scattered apoptotic keratinocytes (Civatte bodies).
Esophageal squamous epithelium with H&E staining at 200x magnification with black arrow showing a Civatte body.
By this time, her symptoms had already become severely debilitating with worsening strictures and narrowing throughout the esophagus, carrying a substantial risk for perforation with continued dilations (Figure
Esophageal mucosal tear after dilatation procedure occurring late in the patient’s course highlighting the potential increased risk of complications with further endoscopic interventions.
Similar to our case, the majority of patients with ELP are middle-aged to elderly females (approximately 87% females with a median age of 61.9) [
On endoscopic examination, ELP may involve the proximal to distal esophagus; however the proximal esophagus (estimated 89% of cases) is most often affected. This is in contrast to reflux disease with distal involvement [
Histopathology may be nondiagnostic in half of cases with interpretations of esophagitis or chronic inflammation. Findings that help support a diagnosis of ELP include a band-like lymphocytic infiltrate involving the superficial lamina propria and basal epithelium along with the presence of Civatte bodies [
As in our case, reflux esophagitis is often initially suspected with initiation of PPI therapy leading to no improvement in strictures [
Treatment generally starts with oral glucocorticoid therapy. The suggested approach has been doses in the range of 40–60 mg for several weeks (patients usually show response within first couple weeks) with a tapered course [
Although it is not certain if LP itself is an independent risk for malignant conversions, there are cases of esophageal squamous cell cancer in those with ELP [
The authors declare that they have no conflicts of interest.