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Case Reports
. 2025 Mar 1;64(5):699-704.
doi: 10.2169/internalmedicine.3769-24. Epub 2024 Aug 1.

Eosinophilic Gastroenteritis with Ascites, Elevated Serum IgG4, and Hypereosinophilic Syndrome: A Manifestation of IgG4-related Disease?

Takao Nagashima  1 , Hiroki Yabe  1 , Naota Okabe  2 , Tsuyoshi Kobashigawa  1
Affiliations
Case Reports

Eosinophilic Gastroenteritis with Ascites, Elevated Serum IgG4, and Hypereosinophilic Syndrome: A Manifestation of IgG4-related Disease?

Takao Nagashima et al. Intern Med. .

Abstract

A 76-year-old woman with persistent diarrhea was referred to our hospital. She had purpura, peripheral eosinophilia (18,177/μL), and an elevated serum IgG4 level (819 mg/dL). Abdominal computed tomography revealed massive ascites and bowel edema. A skin biopsy of the purpura revealed leukocytoclastic vasculitis with prominent eosinophilic infiltration. Biopsies of the gastrointestinal mucosa revealed dense eosinophilic infiltration, indicating eosinophilic gastroenteritis (EG) associated with the hypereosinophilic syndrome. The number of IgG4-positive cells increased in the duodenal mucosa; however, the diagnostic criteria for IgG4-related disease (IgG4-RD) were not met. Whether or not EG with ascites is a manifestation of IgG4-RD warrants further investigation.

Keywords: IgG4-related disease; eosinophilic gastroenteritis; hypereosinophilic syndrome; hypergammaglobulinemia; leukocytoclastic vasculitis.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Clinical picture and histopathology of the skin lesion. A: Purple-to-red rashes, partially palpable, were scattered on the thigh and medial leg. B: The skin histopathology showed leukocytoclastic vasculitis, with many eosinophils infiltrating around vessels (Hematoxylin and Eosin staining, 200×).
Figure 2.
Figure 2.
Contrast-enhanced computed tomography of the abdomen. A: There was bilateral pleural effusion, mild hepatosplenomegaly, and ascites around the liver. The gastric wall was thickened and edematous. B: Wall thickening of the small and large intestines and increased mesenteric density were seen. Enlarged vessels due to splenorenal shunt were also present.
Figure 3.
Figure 3.
Endoscopic findings. A: The duodenal mucosa showed non-specific redness. B: Similar findings were observed in the gastric mucosa. C: Colonoscopy also showed non-specific redness.
Figure 4.
Figure 4.
A biopsy of the duodenal mucosa. A: The number of eosinophils is >40/high-power field [Hematoxylin and Eosin (H&E) staining, 400×]. B: The number of IgG4-positive plasma cells is >10/high-power field (IgG4, 200×). C: Plasmacytic infiltration is predominantly observed in the bottom of the lamina propria (H&E staining, 400×).
See this image and copyright information in PMC

References

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