Eosinophilic esophagitis

Eosinophilic esophagitis, abbreviated EE, is relatively uncommon pathology of the esophagus with some similarities to gastroesophageal reflux disease (GERD).

Eosinophilic esophagitis
Diagnosis in short
Eosinophilic esophagitis. H&E stain.
LM	mucosa with "abundant eosinophils" (see microscopic), basal cell hyperplasia (three cells thick or >15% of epithelial thickness), papillae elongated (reach into the top 1/3 of the epithelial layer)
LM DDx	GERD
Site	esophagus
Associated Dx	atopy, celiac disease
Clinical history	unresponsive to proton pump inhibitors (PPIs)
Symptoms	like GERD
Prevalence	uncommon
Endoscopy	trachealization of esophagus (AKA feline esophagus)
Clin. DDx	GERD

General

• The current thinking is that it is a clinico-pathologic diagnosis.

Clinical:

• Dysphagia - classic presentation.
• Dyspepsia.
  • Often mimics gastroesophageal reflux disease (GERD).

Treatment:

• Avoid exacerbating antigens.
• Topical corticosteroids, e.g. fluticasone.
• Do not respond to proton pump inhibitors.

Biopsies:

• Should be taken from: upper, mid, lower and submitted in separate containers (eosinophilia present through-out-- to differentiate from GERD).

Associations:

• Atopy.
• Celiac disease.
• Oral antigens, i.e. particular foods.
• Familial association.
• Young ~ 35 years old.
• Male > female (3:1).

Gross/endoscopic

• Trachealization; eosphagus looks like trachea.
  • AKA feline esophagus.
• White.

DDx (endoscopic):

• Candida esophagitis

Image

• 

  Trachealization of the esophagus. (WC)

• Trachealization - radiograph (nih.gov).

Microscopic

Features:

• Mucosa with "abundant eosinophils".
• Basal cell hyperplasia.
  • Three cells thick or >15% of epithelial thickness.
• Papillae elongated.
  • Papillae that reach into the top 1/3 of the epithelial layer - definition for GERD.

Notes "abundant eosinophils":

• Criteria for number of eosinophils/area is highly variable; there is a 23X fold variation in published values and only 11% of studies actually define an area (most studies, embarassing for pathologists that understand this issue, only give the number of eosinophils per "HPF")!
  • Interrater variability is low, i.e. good, if the procedure is standardized.
• The most commonly reported cut points are 15, 20 and 24 eosinophils/HPF, without defining HPF.
  • The Foundation Series book says: "> 20/HPF"; onlinepathology sees this definition as garbage, as "HPF" is not defined (see HPFitis).
  • There is a consensus paper that makes note of HPFitis... and then goes on to ignore to whole issue by defining EE as 15/HPF. It blows my mind that the people could be so will fully blind and that the idiotic reviewers didn't understand this.
  • Most resident microscopes at the Toronto teaching hospitals have 22 mm eye pieces and have for their highest magnification objective a 40X. De facto, this means most people in Toronto are using the Liacouras et al. definition.
• Eosinophils may be patchy.

DDx:

• Gastroesophageal reflux disease - no mid and proximal involvement.
• Infectious esophagitis.
• Eosinophilic gastroenteritis.
• Hypereosinophilic syndrome.

Images

• 

  Eosinophilic esophagitis - very high mag. (WC)

• 

  Eosinophilic esophagitis - high mag. (WC)

• Eosinophilic esophagitis (nih.gov).
• EE versus GERD (archivesofpathology.org).

Sign out

ESOPHAGUS, DISTAL, BIOPSY:
- SQUAMOUS MUCOSA WITH BASAL CELL HYPERPLASIA, ABUNDANT INTRAEPITHELIAL EOSINOPHILS, 
  EDEMA, AND PAPILLARY ELONGATION, SEE COMMENT.
- STAINS (PAS-D, GMS) NEGATIVE FOR MICROORGANISMS.
- NEGATIVE FOR INTESTINAL METAPLASIA.
- NEGATIVE FOR DYSPLASIA.

COMMENT:
There are approximately 65 eosinophils per 0.2376 mm*mm (1 HPF). 

Literature valves show a large variation when defining eosinophilic esophagitis 
and frequently use "HPF" as a measure of area, which is not a standardized measure. 
[Am. J. Gastroenterol. 102 (10): 2300–13.] 

Common cut-points are 15 eosinophils/HPF and 20 eosinophils/HPF, where HPF is 
often undefined.

The above findings are suggestive of eosinophilic esophagitis in the proper
clinical context.

Patchy eosinophils

ESOPHAGUS (DISTAL), BIOPSY:
- SQUAMOUS MUCOSA WITH BASAL CELL HYPERPLASIA, INTRAEPITHELIAL EDEMA AND ONLY
  FOCALLY ABUNDANT INTRAEPITHELIAL EOSINOPHILS, SEE COMMENT.
- COLUMNAR EPITHELIUM WITH MODERATE CHRONIC INFLAMMATION, AND PANCREATIC
  ACINAR METAPLASIA.
- NEGATIVE FOR INTESTINAL METAPLASIA.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

COMMENT:
One high power field (field diameter 0.55 mm) has 25 eosinophils. The findings are
compatible with gastroesophageal reflux; however, eosinophilic esophagitis is also a
consideration. Clinical correlation is required.

Literature valves show a large variation when defining eosinophilic esophagitis
and frequently use "HPF" as a measure of area, which is not a standardized measure.
[Am. J. Gastroenterol. 102 (10): 2300 13.]

Common cut-points are 15 eosinophils/HPF and 20 eosinophils/HPF, where HPF is
often undefined.

Histology suggestive

ESOPHAGUS, BIOPSY:
- SQUAMOUS MUCOSA WITH MARKED BASAL CELL HYPERPLASIA, FOCALLY ABUNDANT
  INTRAEPITHELIAL EOSINOPHILS, EDEMA, AND PAPILLARY ELONGATION, SEE COMMENT.
- NEGATIVE FOR INTESTINAL METAPLASIA.
- NEGATIVE FOR DYSPLASIA.

COMMENT:
Focally, there are approximately 35 eosinophils per 0.2376 mm*mm (1 HPF).

The above findings raise the possibility of eosinophilic esophagitis; clinical
correlation is suggested. A re-biopsy including a portion of the proximal esophagus
could be considered.

See also

• Esophagus.
• Eosinophilic colitis.
• Eosinophilic gastritis.