case_images: PMC10751364_01
case_images: Flow cytometric analysis of dihydrorhodamine (DHR) oxidation and expression of gp91
  phox
   in patients. The panel shows a flow cytometric analysis of DHR123 in neutrophils. Blue and red lines indicate unstimulated and phorbol myristate acetate-stimulated neutrophils, respectively.
case_images: fimmu-14-1208590-g001.jpg
case_images: PMC10751364_03_fimmu-14-1208590-g001.jpg
case_images: f1
case_images: The DHR123 flow cytometry assay demonstrated deficient neutrophil oxidative burst activity ( Figure 1 ).
case_images: PMC10751364_02
case_images: Flow cytometric analysis of dihydrorhodamine (DHR) oxidation and expression of gp91
  phox
   in patients. The panel shows a flow cytometric analysis of DHR123 in neutrophils. Blue and red lines indicate unstimulated and phorbol myristate acetate-stimulated neutrophils, respectively.
case_images: fimmu-14-1208590-g001.jpg
case_images: PMC10751364_03_fimmu-14-1208590-g001.jpg
case_images: f1
case_images: The DHR123 flow cytometry assay demonstrated deficient neutrophil oxidative burst activity ( Figure 1 ).
case_images: PMC10751364_03
case_images: Flow cytometric analysis of dihydrorhodamine (DHR) oxidation and expression of gp91
  phox
   in patients. The panel shows a flow cytometric analysis of DHR123 in neutrophils. Blue and red lines indicate unstimulated and phorbol myristate acetate-stimulated neutrophils, respectively.
case_images: fimmu-14-1208590-g001.jpg
case_images: PMC10751364_03_fimmu-14-1208590-g001.jpg
case_images: f1
case_images: The DHR123 flow cytometry assay demonstrated deficient neutrophil oxidative burst activity ( Figure 1 ).
case_images: Endoscopic findings at diagnosis and after treatment of patients. (A, B) Indicate the endoscopic findings of patient 1; (C, D, F) Indicate those of patient 2; and (E, G) Indicate those of patient 3, respectively. (A-E) Indicate findings at diagnosis. (F) Indicates findings after prednisolone (PSL) treatment. (G) Indicates findings after haematopoietic cell transplantation (HCT).
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case_images: PMC10751364_03_fimmu-14-1208590-g002.jpg
case_images: f2
case_images: Patient 1: Colonoscopy revealed non-contiguous and diffuse aphthae throughout the colon ( Figure 2A ). The leopard sign was found slightly in the ascending colon near the ileum ( Figure 2B ). Patient 2: Colonoscopy revealed a diffuse and continuous leopard sign throughout the colon, with slight erythema or erosion ( Figures 2C, D ). Patient 3: Colonoscopy performed before HCT revealed a leopard sign on the colonic mucosa, with slight erythema or erosion ( Figure 2E ). However, in the follow-up colonoscopy 2 months after therapy, the brown dots appeared faded, and the yellowish oedematous mucosa was unchanged ( Figure 2F ). I) occurred on day +14; however, it improved with the adjustment of the tacrolimus dose. In the follow-up colonoscopy on day +43 after HCT, the brown dots appeared to fade, and the yellowish oedematous mucosa was ameliorated ( Figure 2G ).
case_images: Histopathological findings of patients. (A, C, E, F, H, I) depict haematoxylin and eosin staining (A, C, E, and H are magnified x100, and F and J are magnified x400). Arrows indicate pigment-laden CD68-positive macrophages (PLMs). (B, D, G, J) depict anti-CD68 staining [(B, D) are magnified x100, and (G, J) are magnified x400]. (A, B) Indicate samples from patient 1, and (C, D) Indicate samples obtained from patient 2 before prednisolone treatment. (E-G) Indicate samples before haematopoietic cell transplantation (HCT) in patient 3. (H-J) Indicate the samples after HCT in patient 3. The histological findings revealed aggregates of PLMs in the lamina propria. Patient 1 exhibited fewer PLMs in biopsied specimens compared to patients 2 and 3. Following HCT, patient 3's biopsied specimens showed a decrease in PLMs compared to those observed before HCT.
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case_images: PMC10751364_03_fimmu-14-1208590-g003.jpg
case_images: f3
case_images: The histological findings of patients 1-3 revealed aggregates of pale brown pigment-laden CD68-positive macrophages (PLMs) in the lamina propria ( Figures 3A-G ). In patient 1 ( Figures 3A, B ), biopsied specimens showed fewer PLMs than in patients 2 and 3 ( Figures 3C-G ). After HCT, biopsies showed aggregates of granular pigmented macrophages, whose population had decreased compared with that before HCT ( Figures 3H-J ).