A 9-month-old boy was admitted to hospital with acute severe
dehydration because of watery diarrhea. He had a history of low weight for
height, due to chronic intermittent vomiting and diarrhea since 15 days from
birth.
Hypoproteinemia (3.5 g/100 ml), hypoalbuminemia
(1.4 g/100 ml), hypogammaglobulinemia (4.6 g/l) and
lymphocytosis (8.4 G/l) were found. Endoscopy showed an atrophic gastric
mucosa with erosions, without other alterations.
Histology showed a diffuse irregular thickening of the subepithelial
collagen tissue (SCT) (stomach 50 µm, duodenum 12 µm,
colon 12 – 17 µm), erosive epithelium, and
jejunal villous atrophy (grade III).
In spite of 13 years of aggressive treatment (prednisolone
0.5 – 1 mg/kg/d, alternating with budesonide
3 – 4 mg/d, night enteral nutrition, and a
gluten-free diet), clinicopathological disease progression was observed ([Figs. 1], [2]).
Fig. 1 Endoscopic appearance in
10-year-old boy with collagenous gastritis, sprue, and colitis:
a gastric mucosa; b duodenal
mucosa; c right colon; d
transverse colon.
Fig. 2 Histological
appearances: a gastric, showing the antral mucosa with
lymphocytic infiltrate and collagen deposits; b colonic,
showing the collagenous deposits (a and
b, Masson’s trichrome stain
× 400.)
Endoscopy at age 3 showed pseudopolyps with inflammatory areas in
the gastric body, and erythematous mucosal areas in the colon. At age 10, a
thick small tubular stomach, disappearance of gastric folds, and diffuse
atrophic areas (as far as the duodenum), with erythema and multiple small
submucosal nodules were observed. The colonic mucosa had become diffusely pale,
thickened, and nodular, with disappearance of the normal vascular pattern.
Histological examination showed a diffuse atrophic mucosa and an increase in
the SCT (12 – 100 µm) was observed in the
gastrointestinal tract. At age 14, because there was no clinicopathological
improvement, the patient began total parenteral nutrition; this led to
discontinuation of corticotherapy 2 months later and complete clinical
improvement.
Collagenous gastritis, sprue, and colitis are of unknown
pathogenesis and are frequently associated with other disorders
[1]. Diagnosis is on the basis of a strip SCT greater
than 10 µm, with an irregular or focal distribution
[1]
[2]. Symptoms vary according to
the gastrointestinal segment affected [1]
[2]
[3]
[4]
[5]. Collagenous gastritis and colitis are rarely encountered
in pediatrics. Two cases have been reported where these conditions were
associated [4]
[5], but there have
been no previous reports where both entities were associated with collagenous
sprue. As the physiopathology remains unknown, specific treatment is not
available. The long-term outcome is still poorly documented [1]. This case demonstrates the complexity of the
clinicopathological course caused by a diffuse gastrointestinal inflammation
caused by collagenous deposition.
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Endoscopy_UCTN_Code_CCL_1AD_2AD
