Hard to Swallow: A Rare Cause

Abstract

A 66-year-old woman underwent open-access upper endoscopy indicated for a two-month history of odynophagia and dysphagia for solids and liquids, described as difficulty in initiating a swallow followed by the subsequent sensation of food and liquids ''holding up'' in her mid-chest. Past medical history included type 2 diabetes mellitus, hypertension, and dyslipidemia; medications consisted of aspirin, rosuvastatin, losartan, hydrochlorothiazide, omeprazole, glipizide, metformin, oral potassium chloride, thyroid hormone, and insulin glargine. There had been no recent changes in medications or dosages. A focused physical examination prior to the endoscopy was unremarkable. Laboratory evaluation includes a complete blood cell count and basic metabolic panel that also revealed no abnormality. Endoscopy revealed an area of healing confluent ulceration extending over several centimeters in the hypopharynx (Fig. 1a). There was also severe, circumferential, erosive esophagitis that extended 5 cm proximally from the gastroesophageal junction (Fig. 1b). Biopsies of the distal esophagus were taken due to the possibility of a viral etiology of the severe ulceration.Following one of the esophageal biopsies, a prominent mucosal defect concerning for a superficial tear was noted for which an endoscopic hemoclip was deployed; no other complications of endoscopy occurred. In addition to standard anti-reflux measures, the patient was prescribed omeprazole.The esophageal biopsies were reported as showing a focally ulcerated fragment of squamous mucosa with associated fibropurulent material, consisting of mixed inflammatory cells. No nuclear or cytoplasmic inclusions were reported. After endoscopy, her primary care provider prescribed topical treatments for suspected aphthous hypopharyngeal ulceration. Four weeks after endoscopy, she complained of worsening oral pain without improvement of her initial symptoms of odynophagia and dysphagia. An oropharyngeal examination during a clinic visit revealed exudative hypopharyngeal erythema with cracking and ulceration of the buccal mucosa, consistent with the appearance of severe aphthous stomatitis. In view of these findings, buccal biopsies were obtained, which were reported as suprabasal epidermal acantholysis compatible with pemphigus vulgaris (Fig. 2a). Review of the first esophageal biopsy identified a single mucosal fragment with similar suprabasal acantholysis in the buccal specimen, confirming the diagnosis of esophageal pemphigus vulgaris (Fig. 2b). Direct immunofluorescence stain of the buccal specimen revealed a strong, intercellular, intramucosal deposition of IgG and C3, confirming the diagnosis of pemphigus vulgaris (Fig. 3). ManagementAfter the diagnosis of pemphigus vulgaris was confirmed, additional dermatologic evaluation revealed numerous skin and groin lesions, including a violaceous papule on the inferior surface of her abdominal pannus, and papular

Unique causes of esophageal inflammation: a histopathologic perspective

“…Esophagitis dissecans superficialis has also been described in patients with PV . The histologic features of PV involving the esophagus are similar to the skin lesions . The features include the presence of suprabasal epidermal acantholysis and intraepithelial bullae.…”

“…Isolated PV of the esophagus has also been reported . The endoscopic findings of PV in the esophagus include a variety of findings: local mucosal erythema, longitudinal red line, blister, erosions, deep ulcers, exfoliative esophagitis, and stenosis . Esophagitis dissecans superficialis has also been described in patients with PV .…”

“…18 The endoscopic findings of PV in the esophagus include a variety of findings: local mucosal erythema, longitudinal red line, blister, erosions, deep ulcers, exfoliative esophagitis, and stenosis. 16,[18][19][20][21] Esophagitis dissecans superficialis has also been described in patients with PV. 22,23 The histologic features of PV involving the esophagus are similar to the skin lesions.…”

“…22,23 The histologic features of PV involving the esophagus are similar to the skin lesions. 19,21 The features include the presence of suprabasal epidermal acantholysis and intraepithelial bullae. Acantholytic cells in basal layers, resulting in a "tombstone" appearance, are very characteristic.…”