This study calls attention to a new syndrome presenting with gastrointestinal symptoms including
abdominal pain,
vomiting, and/or
hematemesis and endoscopic multiple lesions predominantly in the descending duodenum, without the
skin rash observed in
Henoch-Schonlein purpura. We examined the gastrointestinal mucosa for
IgA deposits in nine children and compared the results with those for three patients with Henoch-Schönlein
purpura. In addition, gastroduodenal biopsy specimens of 11 patients with various diseases were studied as controls for
IgA staining. Intestinal histology showed nonspecific mucosal
inflammation without
vasculitis. In six patients without
rash (67%),
IgA deposition was observed in the capillary wall with the same staining pattern as seen in two patients with
Henoch-Schonlein purpura. Compared with the controls (9%), the positive rate of
IgA deposition was significantly higher in nonrash patients (P < 0.01). Deposited
IgA showed immunoreactivities of polymeric IgAl containing J chain.
IgA deposits were ultrastructually seen along the plasma membranes of the endothelial cells. Overall, the data suggest that
IgA deposition played a pathogenetic role in the gastrointestinal damage in this group of patients presenting primarily with gastrointestinal complaints. Further studies are needed to clarify whether this patient population has a variant of Henoch-Schönlein
purpura or a distinct "
IgA enteropathy."