PMC5398301_01
Physical, magnetic resonance imaging (MRI), and pathological findings. Bluish reticulated patches were noted on the soles and tips of the toes (A), whereas T2-weighted HASTE diagnostic MRI (B) at a slightly more cephalic position from the renal arteries revealed an atherosclerotic plaque consisting of lipids (arrow) and fibrous (arrowhead) tissues in the abdominal aorta. Abnormal fluid-filled lesions (*), which may be ascribed to bilateral simple renal cysts, were also noted. Light microscopy of cutaneous biopsy sections revealed characteristic cholesterol clefts occluding the lumen of the arteriole (C, hematoxylin-eosin stain; the scale bar is indicated).
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Hg. His physical examination revealed livedo reticularis on the soles of his toes and a blue-purple discoloration of all toes bilaterally (Figure 1A), whereas his feet were warm to the touch and the peripheral pulse remained intact. Diagnostic noncontrast-enhanced T2-weighted half-fourier-acquired single-shot turbo spin ecocho (HASTE) magnetic resonance imaging subsequently revealed hypointense areas and bright signals, which were suggestive of an atheromatous plaque consisting of lipids and fibrous tissues, respectively, in the abdominal aorta (Figure 1B). Typical biconvex needle-shaped empty spaces, suggestive of cholesterol clefts, were observed within the lumen of the arteriole in the subcutis (Figure 1C).
Changes in renal parameter values, PT-INR, and percentage of eosinophils. The number "0" was designated as the point of the initial admission, and the patient was discharged on clinical day 51. There was a transient improvement in sCr levels, whereas the irreversible nature of severe renal failure eventually resulted in the initiation of chronic HD despite an increase in the dose of PSL (15 mg/day) combined with 9 sessions of LDL-A. Note that the amounts of urinary excreted proteins, beta2MG, and NAG were almost completely constant during the observation period. The conversion of BUN (mg/dL) to urea (mmol/L) is accomplished by multiplying BUN by 0.357. To convert milligram per deciliter to micromole per liter for sCr, multiply by 88.4.
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After the resumption of an increased dose of PSL (15 mg/day) and the addition of a transient session of LDL apheresis (LDL-A) to the therapeutic regimen, his cutaneous manifestations improved and eosinophilia disappeared; however, severely deteriorated renal function did not recover, and he was finally started on a periodic HD program with favorable volume control (Figure 2).