Temporal Artery Calciphylaxis Presenting as Temporal Arteritis in a Case of Rhinoorbitocerebral Mucormycosis.

Mucormycosis is a rare often fatal opportunistic fungal infection. It is typically described in patients with diabetes in ketoacidotic status and is rare in renal transplant recipients. Calciphylaxis is a rare and highly morbid disease of vascular calcification affecting patients with end-stage renal disease (ESRD). The first case of a renal transplant recipient who was inflicted with both rhinoorbitocerebral mucormycosis and calciphylaxis is reported. A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness. He had undergone renal transplant for ESRD 7 months earlier with resultant immunosuppressive therapy. MRI and nasal biopsy confirmed rhinoorbitocerebral mucormycosis. Immunosuppressive therapy was stopped and antifungal therapy begun. He had orbital exenteration for progressive rhinoorbitocerebral mucormycosis. Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis. Clinical presentation, treatment course, and follow up are discussed.

Calciphylaxis: a case report.

Calciphylaxis or calcific uremic arteriolopathy is characterized by the involvement of the tunica elastica of the reticular or deep subcutaneous dermis, with extravascular and vascular thrombosis calcifications that lead to tissular ischemia. The torso muscles, the lumbar region, and lower limbs are more frequently affected. The pathogenesis is unknown. Calciphylaxis is associated to hyperparathyroidism, chronic renal failure, and diabetes mellitus. The best therapy to date is prevention: early treatment of renal failure, performing a partial parathyroidectomy where necessary, surgical debridement of the necrotic tissue, as well as avoiding the trigger factors such as systemic corticosteroids.

Nanobacteria-caused mitral valve calciphylaxis in a man with diabetic renal failure.

We have found that nanobacteria, recently discovered Gram-negative atypical bacteria, can cause local calciphylaxis on the mitral valve in a setting of high-calcium X phosphorous product in the blood. We present the case of a 33-year-old man with diabetic renal failure on continuous ambulatory peritoneal dialysis who died as a result of multiple brain infarcts due to embolizations from mitral valve vegetations. Systemic calciphylaxis was not present. Spectrometric analysis of the mitral valve vegetations showed that they were composed of calcium phosphate, carbonate apatite form, and fibrin. The electron microscopy of the thrombotic vegetation demonstrated nanobacterium as a nidus for carbonate apatite formation. Investigation for the presence of nanobacteria in the multiple organs involved in systemic calciphylaxis may be of help in elucidating the pathogenesis of this frequently fatal disorder.

Calciphylaxis, proteases, and purpura: an alternative hypothesis for the severe shock, rash, and hypocalcemia associated with meningococcal septicemia.

The hallmarks of severe meningococcal sepsis include the rapid onset of shock, purpuric rash, and metabolic derangement, in particular, hypocalcemia. The severe ecchymoses and purpura associated with meningococcal sepsis are usually attributed to acute thrombotic episodes, attributable to the associated procoagulation disorder. An alternative explanation for the rash is a sudden extravasation of calcium from the intravascular space into the tissues. We will argue that in meningococcal sepsis, cleavage of albumin into fragments by protease(s) occurs and these fragments, along with calcium, cross the endothelium into the interstitium. The fragmentation of albumin and its loss through the endothelium would also provide a more rational explanation for the rapidity of the shock and the hypocalcemia that is so characteristic of the disease.