PMC5642468_01
Radiologic findings in the patient at the age of 9 months. Axial contrast-enhanced computed tomography shows left cervical adenopathy (arrows).
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Figure1
Histopathologically, a cervical lymph node showed changes diagnostic for RDD (Figure 1 and Figure 2).
Immunohistochemistry findings. A cervical lymph node biopsy specimen displays nodal involvement by RDD. Immunohistochemical staining for S-100(+) is positive (A), while staining for CD1a is negative (original magnification, x400) (B).
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Figure2
Histopathologically, a cervical lymph node showed changes diagnostic for RDD (Figure 1 and Figure 2).
Histologic findings at 7 years. The renal biopsy specimen shows enlargement of a glomerulus with mesangial proliferation, thickening of segmental capillary walls, mesangial interposition (thick red arrows) and double contours (thin arrow). Periodic acid-Schiff (PAS) stain (original magnification, x400).
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Figure3
Histologic examination of a renal biopsy specimen disclosed moderate mesangial proliferation, focal thickening of glomerular capillary walls, and mesangial interposition (Figure 3).
Immunofluorescence findings. Immunofluorescence shows intense IgG (A) and C3 (B) deposition in the mesangium and the focally in glomerular capillary walls (circle and arrow) (original magnification, x400). No glomerular IgA, IgM, C1q, or fibrinogen deposition is demonstrated.
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Figure4
Immunofluorescence showed intense IgG, C3, and C4 reactivity in portions of the mesangium and glomerular capillary walls (Figure 4) but no reactivity for IgA and IgM or other complement components such as C1q.
Electron microscopic findings at 7 years. Nodular deposits (arrows) are shown in mesangial, endocapillary, and subepithelial areas (original magnification, x7,000).
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Figure5
Electron microscopy disclosed nodular deposits in mesangial, endocapillary, and subepithelial areas (Figure 5).
Immunohistochemical findings in the kidney upon repeat biopsy. Infiltration of mononuclear cells in the interstitium identified by staining for S-100 (A), CD68 (B), and lysozyme (C). CD1a staining is negative in the same area (magnification, x400) (D).
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Figure6
CD68, and lysozyme was present within the interstitium. CD30 and CD1a reactivity was absent (Figure 6).