PMC9875030_01
Pre-dupilumab histological findings. The figure shows the pre-therapy gastrointestinal eosinophilic infiltrate at different levels: (A-M)(A) (EE 10x) and (B) (EE 20x): glandular and lamina propria eosinophilic infiltrate in duodenal mucosa (arrow). (C) (EE 5x), (D) (EE 10x), (E) (EE 20x), (F) and (G) (EE 40x): gastric eosinophilic infiltrate (>50xHPF) either in glands and in lamina propria and in muscularis mucosae with a micro-erosive intraglandular pattern (arrow). (H, L, M) (EE 40x) and (I) (EE 10x): eosinophilic intraepithelial infiltrate (35-40xHPF).
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He started presenting gastrointestinal symptoms by the age of 3, particularly loss of appetite, nausea, heartburn, vomiting and recurrent diarrhea, thus he was diagnosed with eosinophilic gastroenteritis by biopsy ( Figure 1 ) and subsequently identified as IPEX syndrome because of the presence of a hemizygous mutation in FOXP3 gene (c.543C>T (p.S181S) in the exon 5). Histology obtained from endoscopic biopsies showed diffuse gastrointestinal eosinophilic infiltration ( Figure 1 ).
Post-dupilumab histological findings. Eosinophilic infiltrate strongly decreased after biological therapy. Figure shows 9 months post-therapy histology at different levels: n-u: (N) (EE20x), (O) and (P) (EE 40x) duodenal and (Q) (EE20x), (R) (EE20x) and (S) (EE40x) gastric strongly decreased granulocitic infiltrate (<10xHPF without aggressive infiltrating pattern) (T, U) (EE 40x): less than 4-5 intraepithelial esophageal eosinophils xHPF can be detected (arrows).
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Moreover, endoscopic procedures, repeated after 9 months, showed a global depletion of eosinophils in the tissues ( Figure 2 ).