PMC5173500_01
The renal biopsy findings. A light micrograph of the glomerulus (A) shows no glomerular changes (Periodic acid-Schiff stain), while an electron micrograph of a portion of the glomerulus (B) demonstrates diffuse foot process effacement (asterisks) and vacuolation. En: glomerular endothelial cell. The scale bar and scale are indicated in each panel.
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An immunohistochemical analysis failed to reveal any immune complex deposits, while electron microscopy showed a flattening of the foot processes of the glomerular visceral epithelial cells without any apparent electron dense deposits within the glomerular basement membrane or mesangial area, which was consistent with minimal change disease (MCD) (Fig. 1).
The clinical course of the patient. Serial changes in several clinical parameters and therapeutic regimens during the observation period are shown. Clinical day 0 is designated as the point of admission. The patient's renal function steadily worsened despite treatment with glucocorticoids, immunosuppressants, and LDL-A, and a HD was subsequently performed three times a week from clinical day 64. Note that the patient was switched from MZB to CyA on clinical day 71.
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Finally, after treatment with oral cyclosporine A (CyA) (150 mg/day) for approximately two weeks and five sessions of double filtration plasmapheresis (DFPP), his urine output began to increase (Fig. 2).
An early skin lesion in the left leg showed a palpable painful petechial rash (A). It developed into dusky violaceous purpuric plaque with overlying eschars (B) and became ulcerative with surrounding erythema (C) at one and two months after the onset of symptoms, respectively. The fully healed wound on same aspect was confirmed in May 2013 (D). A skin biopsy specimen showed a small artery with medial calcification accompanied by edematous intimal thickening (E, Hematoxylin and Eosin staining). A scale bar is shown. A bone scan performed in the middle of February 2013 demonstrated the superficial location of radiotracer activity in the subcutaneous region of the lower extremities although the uptakes were most prominent in the right thigh (F).
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About one month after the last HD session, he started to complain of several small firm and painful nodules with purple discoloration on the lower extremities (Fig. 3A). A skin biopsy revealed arterior calcium deposits with intimal proliferation (Fig. 3E). The lesions became much larger and ulcerative with surrounding erythema over the next two to five weeks (Fig. 3B and C), and a bone scan revealed an increased tracer accumulation in the soft tissue of both legs (Fig. 3F). The wounds were confirmed to have fully healed ten months later (Fig. 3D).