TWO CASES OF PSEUDOXANTHOMA ELASTICUM WITH RENAL INVOLVEMENT (CASE REPORT)

Pseudoxanthoma elasticum is a rare, hereditary, multisystemic disease affecting the skin, eye, and cardiovascular system. Renal involvement is uncommon. We describe two cases of pseudoxanthoma elasticum (PXE) in two women with distinctive skin lesions and nephrocalcinosis that renal ultrasonography showed a characteristic pattern of dotted increased echogenicity in the corticomedullary junction. One of them had IgA nephropathy that was proven by kidney biopsy. Skin biopsy and fundus examination confirmed the diagnosis. Early diagnosis of PXE is important for minimizing systematic complications and informing the other family members through genetic counseling. Our case with PXE and IgA nephropathy is the first case report.