Within weeks of developing necrotic digits, the patient became incapacitated by severe lower extremity pain that was only partially controlled by high-dose fentanyl patches. Topical mupirocin ointment has been the only dermatologic treatment throughout the progression of this disease. The patient currently waits for a hepatorenal transplant. I primary hyperoxaluria is a result of an inborn error of glyoxylate metabolism that leads to excessive glyoxalate conversion to oxalate, a renally excreted endogenous metabolite. 3 Oxalate precipitates as calcium oxalate throughout the body, in particular in the bones, myocardium, and smooth muscle walls of arterial blood vessels.
Cutaneous lesions are distinctive and herald a moderately advanced stage of disease.
Crystal deposition in the kidneys leads to renal insufficiency and failure. Because dialysis filtration is unable to match oxalate production, disease progression subsequently accelerates at a marked rate, leading to death by the third to fourth decade of life. Even with dialysis support, hepatic deposition eventually causes liver failure. Liver or liver-kidney transplantation remains the only definitive treatment. Singh S, Tai C, Ganz G, et al.