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 1-3). Initial examinations revealed strictures in the lower third of the esophagus. Later, strictures were discovered in both the proximal and mid-esophagus. Strictures ranged in diameter within 9-14 mm.
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 4 and 5) raising strong consideration of ELP. After a multidisciplinary review of these findings along with her consistent history, a diagnosis of ELP was made.
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 6). The patient ultimately made a decision to undergo a minimally invasive esophagectomy for definitive management with acute and chronic inflammation seen on surgical pathology. Her symptoms significantly improved in the months following her surgery. However, she gradually developed symptoms of dysphagia, abdominal cramping, nausea, emesis, and weight loss. This was felt to be secondary to delayed gastric emptying and a mild anastomotic stricture. She underwent Botox injection into the pylorus along with therapeutic dilation and triamcinolone acetonide injection to stricture. Her symptoms improved after these interventions.