Eosinophilic esophagitis

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

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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.
• Eosinophil.