Esophagus
Esophagitis
Eosinophilic esophagitis


Topic Completed: 20 May 2020

Minor changes: 20 May 2020

Copyright: 2003-2020, PathologyOutlines.com, Inc.

PubMed Search: Eosinophilic esophagitis [title] pathology review[ptyp] loattrfree full text [sb]

Ryan C. Braunberger, M.D.
Joshua A. Hanson, M.D.
Page views in 2019: 12,198
Page views in 2020 to date: 9,708
Cite this page: Braunberger RC, Hanson JA. Eosinophilic esophagitis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophaguseosinophilic.html. Accessed September 26th, 2020.
Definition / general
  • Multifactorial, chronic eosinophilic inflammatory condition affecting the esophagus in both adult and pediatric patients that occurs in the absence of identifiable secondary causes (Med Clin North Am 2019;103:29)
  • Characterized by a chronic immune reaction to environmental and food allergens leading to a deficient esophageal mucosal barrier (Arch Pediatr 2019;26:182)
Essential features
  • Major diagnostic criteria of ≥ 15 eosinophils per high powered field (40x magnification) in the proper clinical context (Arch Pediatr 2019;26:182)
Terminology
  • Idiopathic or primary eosinophilic esophagitis
  • Chronic atopic inflammatory disease of the esophagus
  • Allergic or atopic esophagitis
ICD coding
  • ICD-10: K20.0 - eosinophilic esophagitis
Epidemiology
Sites
  • Can diffusely affect the entire length of the esophagus
Pathophysiology
  • Intraepithelial eosinophils recruited via chemotaxis act as antigen presenting cells (APCs) recruiting T cells, activating mast cells and activating basophils (Arch Pediatr 2019;26:182)
  • Eosinophils secrete a cationic protein eosinophilic peroxidase (EPO) and major binding protein (MBP) causing cell damage leading to the symptom of esophageal dysmotility (Arch Pediatr 2019;26:182)
    • Concentration of eosinophilic peroxidase (EPO) correlates to increased symptomology, not the number of intraepithelial eosinophils (Arch Pediatr 2019;26:182)
  • Recruited T cells differentiate into Th2 T cells and secrete IL4, IL5 and IL13 (Arch Pediatr 2019;26:182)
Etiology
  • Environmental
    • Food allergens
    • Hygiene and bacterial dysbiosis hypothesis
      • Improved hygiene conditions lead to reduced bacterial exposures and alterations in the microbiota, which may alter mucosal permeability (World J Gastroenterol 2019;25:4598)
    • Gastroesophageal reflux disease (GERD) leads to increased mucosa permeability to food allergens
    • Proton pump inhibitor (PPI) usage
      • Reduced acid secretion downregulates digestive enzyme activation
      • Food antigens are not degraded, increasing the immune response to these large proteins (World J Gastroenterol 2019;25:4598)
  • Genetic / familial
    • Genetic variants at multiple loci have been shown to increase the risk of development of atopic diseases, including eosinophilic esophagitis and asthma
      • Genes found with alterations in patients with eosinophilic esophagitis:
        • CCL26 - encodes eotaxin3
        • Thymic stromal lymphopoietin (TSLP)
        • Filaggrin (FLG)
        • Desmoglein1 (DSG1)
        • STAT6
        • Calpain 14 (CAPN14)
        • CRLF2
Clinical features
Diagnosis
  • Eosinophilic esophagitis is a clinicopathologic diagnosis and findings should be considered in combination and not independent of each other
  • Current diagnostic modalities
    • Esophagogastroduodenoscopy (endoscopy)
      • Endoscopy may show many characteristic but not pathognomonic changes
        • Major criteria: mucosa edema, white exudate, longitudinal furrows, circular esophageal rings (also called pseudotrachea), esophageal stenosis (Gut 2013;62:489)
        • Minor criteria: feline esophagus (concentric mucosal rings observed with some types of motility that disappear with air insufflation), narrow caliber esophagus, crêpe paper esophagus, mucosal laceration or fragility with passage of instruments before dilation (Gut 2013;62:489)
      • Up to 25% of patients have normal endoscopic findings (World J Gastroenterol 2019;25:4598)
    • Esophageal biopsies are required for diagnosis
    • Histology
      • Major criteria: ≥ 15 eosinophils per high powered field (40x magnification) (Arch Pediatr 2019;26:182)
      • Minor criteria: extreme basal zone hyperplasia with papillary hyperplasia, eosinophils concentrated in the surface epithelium as opposed to the base, eosinophilic microabscesses, eosinophil degranulation, surface desquamation, lamina propria fibrosis (Arch Pediatr 2019;26:182)
  • Noninvasive diagnostic procedures being tested
    • Esophageal string test (EST) (Am J Gastroenterol 2019;114:1614)
      • Currently in clinical trials
      • 1 hour test that can distinguish active from inactive disease in both children and adults
      • Minimally invasive way to monitor disease
    • Cytosponge (Curr Treat Options Gastroenterol 2019;17:48)
      • Mesh sponge tethered by a string that is compressed in a dissolvable capsule
      • Capsule is swallowed and then withdrawn through the mouth
      • Sensitivity of 75% and specificity of 86%
    • MicroRNA expression (Curr Treat Options Gastroenterol 2019;17:48)
      • Esophageal brushings obtained via endoscopy or via nasogastric tube are analyzed for the presence and quantity of eosinophil derived neurotoxin which is overexpressed in eosinophilic esophagitis
Radiology description
Case reports
  • 11 year old boy with allergic rhinitis and chest pain (Clin Endosc 2019;52:606)
  • 34 year old man with refractory heartburn following 2 month trial of proton pump inhibitor and intermittent dysphagia (CMAJ 2016;188:893)
  • 40 year old Caucasian woman with eosinophilic esophagitis and spontaneous transmural esophageal perforation (J Med Case Rep 2019;13:275)
Treatment
  • Treatment revolves around the 3 D’s - drugs, diet and dilation
  • Drugs
    • Proton pump inhibitors:
      • First line therapy
      • Generally used in combination with dietary modifications as there is a high association with food allergens
    • Corticosteroids:
      • Second line therapy
      • Topical or systemic (systemic reserved for patients with severe symptoms)
  • Diet
    • Dietary modification due to the role that food allergens play in the pathogenesis of the disease
      • Elemental diet
      • Elimination diets
        • Guided by allergy testing
        • Empiric elimination diets
  • Dilation
    • Treatment for esophageal strictures and narrowing
      • Improves symptoms of dysphagia only
      • A meta analysis showed the risk of perforation from dilation procedures in eosinophilic esophagitis was 0.3% (Med Clin North Am 2019;103:29)
Clinical images

Contributed by Dr. Pankaj Vohra

Furrows

Tear

Gross description
  • Major criteria (Gut 2013;62:489)
    • Mucosa edema
    • Exudate or white plaques
    • Longitudinal furrows
    • Circular esophageal rings (pseudotrachea)
    • Esophageal stenosis
  • Minor criteria (Gut 2013;62:489)
    • Feline esophagus
      • Concentric mucosal rings observed with some types of motility that disappear with air insufflation
    • Narrow caliber esophagus
    • Crêpe paper esophagus
      • Mucosal laceration or fragility with passage of instruments before dilation
Microscopic (histologic) description
  • Major criterion: ≥ 15 eosinophils per high powered field (40x magnification) present in the squamous mucosa (Arch Pediatr 2019;26:182)
  • Minor criteria: eosinophils concentrated in the surface epithelium as opposed to the base, extreme basal zone hyperplasia, eosinophilic microabscesses, eosinophil degranulation, surface desquamation, lamina propria fibrosis (Arch Pediatr 2019;26:182)
Microscopic (histologic) images

Contributed by Ryan C. Braunberger, M.D. and Joshua A. Hanson, M.D.

Extreme basal cell hyperplasia

Surface concentration of eosinophils

Eosinophilic microabscess

Eosinophil degranulation

Virtual slides

Images hosted on other servers:

Eosinophilic esophagitis

Perforated esophagus with eosinophilic esophagitis

Videos

Endoscopic features of eosinophilic esophagitis

Sample pathology report
  • Esophagus, mid, biopsy:
    • Eosinophil rich esophagitis (> 50 eosinophils per single high powered field, maximum count) (see comment)
    • Comment: The biopsy demonstrates marked reactive basal zone hyperplasia with increased eosinophils. The eosinophils have a top heavy distribution and few eosinophilic microabscesses are identified. These features are consistent with eosinophilic esophagitis in the proper clinical context.
Differential diagnosis
  • Gastroesophageal reflux disease (GERD):
    • Triad of findings includes:
      • Hyperplasia of lamina propria papillae (greater than two thirds of the epithelial thickness)
      • Mild basal zone hyperplasia (approximately 15 - 25% of the epithelial thickness)
      • Mildly increased intraepithelial eosinophils concentrated at the base of the mucosa as opposed to the surface (usually < 10 per high power field though counts are not pathognomonic for either condition) (Arch Pediatr 2019;26:182)
    • Eosinophil counts in GERD are generally higher in distal esophageal biopsies compared with more proximal biopsies (this is reversed in eosinophilic esophagitis but is not pathognomonic)
    • No surface eosinophilic microabscesses
    • No lamina propria fibrosis
    • Other intraepithelial inflammatory cells seen in some but not all cases include lymphocytes and neutrophils
  • Eosinophilic gastroenteritis:
    • Affects any portion of the gastrointestinal tract with the stomach and small bowel being the most common locations (Lancet Gastroenterol Hepatol 2018;3:271)
    • Symptoms such as diarrhea, intestinal obstruction and ascites are not common in eosinophilic esophagitis
    • When seen in the esophagus, can be an exact histologic mimic of eosinophilic esophagitis so clinical presentation and correlation with other mucosal biopsies is essential
Board review style question #1



A 12 year old boy with a history of seasonal allergies and asthma complains of dysphagia and abdominal pain. Endoscopic biopsies reveal increased eosinophils in the esophageal squamous mucosa. What feature pictured in the image favors eosinophilic esophagitis over GERD as the most likely diagnosis?

  1. Increased mitotic activity
  2. Intercellular edema
  3. Scattered intraepithelial lymphocytes
  4. Superficial concentration of eosinophils
Board review answer #1
D. Superficial concentration of eosinophils

Comment Here

Reference: Eosinophilic esophagitis
Board review style question #2
A 36 year old man presents to clinic with the chief complaint of intermittent dysphagia and heartburn that has not responded to over the counter medications. Endoscopy was notable for white plaques in the esophagus, longitudinal furrows and pseudotrachea. Biopsies taken during endoscopy show increased eosinophils in the distal and proximal specimens. Which chemokine producing immune cell plays an essential role in eosinophil infiltration and activation?

  1. B cells
  2. Eosinophils
  3. Mast cell
  4. Th2 T cells
Board review answer #2
D. Th2 T cells

Comment Here

Reference: Eosinophilic esophagitis
Back to top
Image 01 Image 02