Crohn's disease of the esophagus is rare, and it is very unusual for i
t to be located only in the esophagus. We report a case of Crohn's dis
ease confined to the esophagus in a 26-year-old female. The patient wa
s admitted because of progressive dysphagia, odynophagia and weight lo
ss. A barium-swallow examination showed an irregular narrowing of the
esophagus below the level of the aortic arch which was 15cm long, with
marginal ulcers and a pseudopolypoid appearance of the mucosa; a comp
uted tomographic scan of the thorax revealed a thickened esophageal wa
ll. Esophagoscopy revealed an esophageal stricture 25cm distal to the
incisor teeth, 2mm in diameter, with ''punched out'' ulcers and pseudo
polypoid mucosa. Endobiopsy specimens showed chronic lymphocytic infil
tration into the corion in the absence of neutrophils, basal-cell hype
rplasia and elongation of the stromal papillae.The patient underwent a
n esophagectomy through a combined cervico-abdominal approach followed
by a cervical esogastrostomy. The specimen was 18cm long, the thickne
ss of the wall was 1.7cm with fibrosis involving all layers of the eso
phageal wall and a cobblestone appearance of the mucosa. A heavy lymph
oplasmocytic infiltrate extended from the mucosa deep into the muscula
ris, fibrosis and granulomas were found transmurally. Crohn's disease
of the esophagus is a rare and specific entity which can present in va
rious ways; strictures resembling those from reflux esophagitis or a t
umor are common. Diagnosis may be suggested by the presence of a chron
ic lymphocytic infiltrate with or without non-caseating granulomas, an
d no histologic evidence of chronic reflux esophagitis.