PMC9309483_01
Clinical image of the dystrophic epidermolysis bullosa patient and histology from renal biopsy. (A) Skin scarring on feet, pseudosyndactyly and nail dystrophy. (B) H&E staining. (C) Electron micrography image. (D) IgA immunofluorescence staining. (E) C3 immunofluorescence staining.
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F1
The patient had limited mouth opening, irregular dentition, nail dystrophy, pseudosyndactyly, and flexion deformities of interphalangeal joints due to progressive scarring of the extremities (Figure 1A). It revealed vacuolar and granular degeneration of tubular cells, partial atrophy of tubule, interstitial edema, and mild interstitial fibrosis with scattered clusters of lymphocyte and mononuclear cell infiltration in interstitium accompanied by vascular wall thickening of interstitial vessels (Figure 1B). The depositions of IgA, C3, and fibrinogen were observed in mesangial area upon immunofluorescence (IF) staining (Figures 1D,E). High density electron dense deposits were observed in mesangium, and occasionally found in subepithelium and subendothelial lesions (Figure 1C).
Twenty four hour urinary protein over the treatment course. The arrow indicated the time points when skin lesions worsened.
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F2
Twelve months after initiation of therapy, urinary occult blood became negative. 24-h urinary protein decreased to 195.5 mg (Figure 2).