A forty year-old woman was admitted to the hospital in order to evaluate the cause of pedal edema and proteinuria. The physical examination revealed almost normal except for hypoplasia of nails of thumbs, index fingers and middle fingers on both side. Laboratory findings coincided with those of a nephrotic syndrome, but the histology of renal biopsy specimens indicated the characteristic findings of the nail-patella syndrome (onyco-osteodysplasia), namely massive collagenous accumulation at the site of endothelial layer of the glomerular basement membrane. Only the onychodyspalsia was seen in this case though the nail-patella syndrome is a hereditary disorder characterized by nail dysplasia and multiple osseous abnormalities principally affecting the elbows, knees and conical illiac horns. This case seemd to be a very rare one in respect to the incomplete nail-patella syndrome. There were only 13 cases of nail-patella syndrome hetherto reported and 6 cases among them presenting nephropathy in Japan. Clinical and morphologic study of this case led us to more the value of utilization of a renal biopsy on the case of steroid resistant nephrotic syndrome.
