Medical Journal of The Islamic Republic of Iran (MJIRI)

  Abstract

  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.