Eosinophilic Esophagitis

Definition

• Marked eosinophil infiltration restricted to the esophagus

Alternate/Historical Names

• Allergic esophagitis

Diagnostic Criteria

• Intraepithelial eosinophils at least 15/HPF in any one field (Furuta)
• 6-14/HPF is considered indeterminate
• The specificity of such cutoffs has been challenged (Rodrigo)
• Eosinophils may be diffuse or in clusters
• Microabscess is defined as a cluster of 4 or more
• Eosinophils often located in superficial layers of mucosa
• Superficial epithelium containing sheets of eosinophils may slough
• Eosinophilic infiltrates must not be present in rest of GI tract
• Generalized GI infiltrates should suggest eosinophilic gastroenteritis
• Peripheral eosinophilia may be present but usually <1500/ml
• Report should contain the following
• Number of eosinophils/HPF
• Presence or absence of microabscesses
• Distribution: diffuse or accentuated at surface
• Presence or absence of squamous hyperplasia
  • Papillary or basal cell

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting/updates : 11/11/09, 11/11/11

Differential Diagnosis

Eosinophilic GI disorders by location

Eosinophils may be numerous in other diseases

• Food impaction must be ruled out clinically
• Lodging of medicinal agents (pill esophagitis) must be ruled out clinically
• Drug related must be ruled out clinically
• Hypereosinophilic syndrome
  • Requires >1500 eosinophils / microliter
• Parasitic disease must be ruled out by stool examination
• Crohn disease
  • May contain many eosinophils in the infiltrate but they do not predominate
  • Very rare in esophagus
• Milk protein enteropathy must be ruled out clinically especially in infants
• Systemic diseases
  • Churg Strauss vasculitis
  • Pemphigoid
  • Polyarteritis nodosa
  • Scleroderma
  • Stevens-Johnson syndrome
• Graft versus host disease

Clinical

• Peak age 20-40 years
• Range 6 months to 89 years
• Rare under 1 year of age
• Frequently associated with allergy, asthma, atopy
• Peripheral eosinophilia may be present but usually <1500/ml
• May have dysphagia, focal strictures and food impaction
• Endoscopic findings
• Longitudinal furrows
• Multiple rings (feline esophagus)
• Fragile and inelastic (crepe paper esophagus)
• White exudates
• Rich in eosinophils
• Focal strictures and long segment strictures
• Failure to thrive in infants
• Pediatric
• May respond to elemental (amino acid) diet or dietary restrictions
• May respond partially or not at all to anti-reflux therapy
• May respond to oral steroids
• Adult
• Increasingly diagnosed in patients refractory to anti-reflux therapy with normal pH
• May respond to antihistamines or topical steroids

Bibliography

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• Chang F, Anderson S. Clinical and pathological features of eosinophilic oesophagitis: a review. Pathology. 2008 Jan;40(1):3-8.
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• Liacouras CA, Furuta GT, Hirano I, Atkins D, Attwood SE, Bonis PA, Burks AW, Chehade M, Collins MH, Dellon ES, Dohil R, Falk GW, Gonsalves N, Gupta SK, Katzka DA, Lucendo AJ, Markowitz JE, Noel RJ, Odze RD, Putnam PE, Richter JE, Romero Y, Ruchelli E, Sampson HA, Schoepfer A, Shaheen NJ, Sicherer SH, Spechler S, Spergel JM, Straumann A, Wershil BK, Rothenberg ME, Aceves SS. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol. 2011 Jul;128(1):3-20.