1/4. Steroid-responsive pleuropericarditis and livedo reticularis in an unusual case of adult-onset primary hyperoxaluria.We present a case of a 54-year-old woman with rapidly progressive renal failure of uncertain origin, who developed pleuropericarditis and livedo reticularis 6 weeks after initiation of hemodialysis (HD). The presentation with acute renal failure, the development of serositis, and the dramatic clinical response to empiric steroid therapy initially suggested the diagnosis of a systemic inflammatory disorder or vasculitis. Renal biopsy, performed 3 days after presentation, suggested crystal deposition disease, and subsequent investigations, using both dialysate oxalate concentrations and liver biopsy, led to the diagnosis of primary hyperoxaluria (PH). We discuss this atypical adult presentation of PH and propose a role for the use of steroids in the management of the acute inflammatory symptoms of oxalosis. We also briefly discuss the current medical management of patients with PH, including transplantation.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
2/4. Clinical and histologic features of primary oxalosis.Primary oxalosis should be considered in patients with multisystem disease of the kidneys, heart, peripheral vasculature, and skin. Crystalline deposits can lead to nephrolithiasis with kidney failure, complete heart block, peripheral vasospasm, and livedo reticularis, as in our patient. Crystals were first observed in the myocardial biopsy specimen and then identified as calcium oxalate in skin from an area of livedo reticularis.- - - - - - - - - - ranking = 0.4keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
3/4. livedo reticularis and peripheral gangrene associated with primary hyperoxaluria.We describe a 38-year-old man who developed livedo reticularis and peripheral gangrene after bilateral nephrectomy. Pathologic evaluation revealed extensive crystalline deposits within the walls of subcutaneous blood vessels. The diagnosis of primary hyperoxaluria was established. This case demonstrates that the vasculopathy of primary oxalosis can mimic systemic necrotizing vasculitis.- - - - - - - - - - ranking = 0.36818304510415keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
4/4. Primary hyperoxaluria in an adult with renal failure, livedo reticularis, retinopathy, and peripheral neuropathy.We present the case of a young woman who developed renal failure of unknown cause, and after 2 months of maintenance hemodialysis developed livedo reticularis, retinopathy, and peripheral sensory neuropathy. The patient was subsequently shown to have primary oxalosis type I, a rare autosomal recessive error of metabolism characterized by accumulation of calcium oxalate crystals in the kidneys, eyes, skin, and other organs. Intravascular obstruction, caused by deposition of calcium oxalate crystals in cutaneous arterioles, is thought to be responsible for the ischemic livedo reticularis lesions observed in this patient. A method is described for measuring serum glycolate by isotope dilution gas chromatography-mass spectrometry (GC-MS). An approach to the diagnosis and management is also briefly mentioned.- - - - - - - - - - ranking = 1.2keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |