A 50-year-old woman presented with dysphagia since 1 week. She did
not drink alcohol and there was no history of systemic disease, including
dermatologic, immunologic, or genetic disease. The initial laboratory findings,
complete blood count, and serum biochemistries were normal, except the serum
vitamin B12 level, which was 52 pg/mL (normal range:
126 – 505 pg/mL). Upper endoscopy revealed
pangastritis with whitish strips and pseudomembranes on the esophageal mucosa,
which peeled off similarly to eosinophilic esophagitis or a lesion of
dermatologic origin ([Fig. 1]).
Fig. 1 The whitish strips with
extensive sloughing of the esophageal mucosa.
The lesions, which presented as discrete patches starting in the
upper esophagus, extended diffusely through the entire esophagus. While the
gastric biopsy samples showed features of atrophic gastritis, the esophageal
biopsy samples were interpreted as parakeratosis ([Fig. 2]).
Fig. 2 Histologic section of
the esophagus showing a dense layer of parakeratosis (hematoxylin and eosin
stain).
Serum antiparietal antibodies were also positive. The patient was
diagnosed as having early-stage pernicious anemia and esophageal parakeratosis.
Replacement therapy with vitamin B12 injections was followed by
prompt resolution of the dysphagia, and a repeat endoscopy showed complete
healing of the esophageal mucosa ([Fig. 3]).
Fig. 3 Normal esophageal mucosa
seen at the control endoscopy 5 days later.
The control biopsy samples showed only minimal parakeratosis despite
the short-term therapy ([Fig. 4]).
Fig. 4 Reduction in epithelial
parakeratosis after therapy (hematoxylin and eosin stain).
Diffuse esophageal parakeratosis is a rare endoscopic diagnosis and
is associated with conditions such as tylosis, mucosal hyperkeratosis syndrome,
pachyonychia congenita, ethanol exposure, duodenal reflux, riboflavin
deficiency, and zinc deficiency [1]
[2]
[3]
[4]
[5]
[6]. Following this first report of
pernicious anemia due to vitamin B12 deficiency leading to
esophageal parakeratosis, we recommend adding it to the long list of etiologic
factors of this condition. Our patient presented with the sole symptom of
dysphagia, that is without the established findings of pernicious anemia, such
as low hemoglobin, other cytopenias, neurologic findings, and hemolysis. Like
the majority of reported cases of esophageal parakeratosis due to nutritional
deficiencies, our patient’s symptoms and signs also resolved after
replacement of the specific deficiency [7].
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