Background

For many years, this disease was thought to be a variant of reflux disease. It is now known to be a distinct condition and thought to be an allergic reaction, predominately occurring in children. The gold standard for diagnosis is a normal 24 hour pH probe study despite severe esophagitis.

Outline

Epidemiology
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Differential Diagnosis
Prognosis and Treatment
Commonly Used Terms

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	EE
INCIDENCE	Increasingly common
AGE RANGE-MEDIAN	All ages but more common in children
SEX (M:F)	May have slight male predominance

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
Asthma	>70% Family history of allergy in 30-50% Peripheral esoinphilia in 50% Abnormal skin testing in >70%

 

PATHOGENESIS	CHARACTERIZATION
Probable allergic basis	Etiologic agent remains unknown Suspected food allergen or inhaled or swallowed airborne allergen Intraepithelial Langerhans cells recognize antigen and stimulate T cell proliferation with production of eosinophil cytokines

 

LABORATORY/RADIOLOGIC/ OTHER TESTS	CHARACTERIZATION
RADIOLOGIC
LABORATORY MARKERS
24 hour pH probe	Normal study

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL	Endoscopy shows characteristic punctate white surface dots associated with erythema, loss of vascular pattern, ulcers, or ringed trachea-like appearance
The spectrum of pediatric eosinophilic esophagitis beyond infancy: a clinical series of 30 children. Orenstein SR, Shalaby TM, Di Lorenzo C, Putnam PE, Sigurdsson L, Kocoshis SA. Division of Pediatric Gastroenterology, University of Pittsburgh School of Medicine, and Children's Hospital of Pittsburgh, Pennsylvania 15213, USA.	Am J Gastroenterol 2000 Jun;95(6):1422-30 Abstract quote OBJECTIVES: Eosinophilic esophagitis, previously confused with esophageal inflammation due to gastroesophageal reflux, has recently begun to be distinguished from it. We undertook this analysis of our large series of children with the condition to clarify its spectrum: its presenting symptoms; its relation to allergy, respiratory disease, and reflux; its endoscopic and histological findings; and its diagnosis and therapy. METHODS: We analyzed the details of our clinical series of 30 children with eosinophilic esophagitis, defining it as > or =5 eosinophils per high power field in the distal esophageal epithelium. Retrospective chart review was supplemented by prospective, blinded, duplicate quantitative evaluation of histology specimens, and by telephone contact with some families to clarify subsequent course. Presentation and analysis of the series as a whole is preceded by a case illustrating a typical presentation with dysphagia and recurrent esophageal food impactions. RESULTS: Presenting symptoms encompass vomiting, pain, and dysphagia (some with impactions or strictures). Allergy, particularly food allergy, is an associated finding in most patients, and many have concomitant asthma or other chronic respiratory disease. A subtle granularity with furrows or rings is newly identified as the endoscopic herald of histological eosinophilic esophagitis. Histological characteristics include peripapillary or juxtaluminal eosinophil clustering in certain cases. Association with eosinophilic gastroenteritis occurs, but is not common. Differentiation from gastroesophageal reflux disease is approached by analyzing eosinophil density and response to therapeutic trials. Therapy encompasses dietary elimination and anti-inflammatory pharmacotherapy. CONCLUSION: Awareness of the spectrum of eosinophilic esophagitis should promote optimal diagnosis and treatment of this elusive entity, both in children and in adults.

 

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL	NOTE: These changes are indicative of the disease only after documented anti-GERD therapy Large numbers of intraepithelial eosinophils (>20/hpf) with extensive eosinophil degranulation Preferential localization of eosinophils in upper half of epithelium Surface eosinophil microabscesses Surface slough of necrotic keratinocytes admixed with eosinophils Long linear extent of esophagitis Pathologic changes more severe in upper and mid-esophagus than near GE junction Increase in intraepithelial CD3 and CD8 lymphocytes and CD1a antigen presentin cells
VARIANTS

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
GERD
Infections
Pill-induced damage
Damage secondary to chemo- and radiation therapy
Allergic or eosinophilic esophagitis

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSTIC FACTORS
TREATMENT	Dietary restrictions Systemic corticosteroids

Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Dig Dis Sci 1993;38:109-116
J Pediatr Gastroenterol Nutr 1998;27:90-93
J Pediatr Gastroenterol Nutr 2000;30:S28-S35
Am J Gastroenterol 2000;95:1422-1430
Am J Gastroenterol 2000;95:1572-1575

Esophagus

Last Updated 11/29/2001