1/7. calciphylaxis with peau d'orange induration and absence of classical features of purpura, livedo reticularis and ulcers.calciphylaxis is an ill-defined syndrome that is commonly associated with chronic renal failure. Its heterogeneous clinical features include painful livedo reticularis-like purpuric patches and plaques, vesicles, irregularly shaped ulcers, and black eschars. Despite demonstration of extensive vascular arteriolar calcification in this syndrome, its exact pathogenesis remains unknown. Here, we report a case of calciphylaxis presenting with indurated plaques without the usual clinical picture of livedo reticulate purpura, ulcers or necrotic eschars. This case provides an opportunity to review the clinical spectrum of calciphylaxis and to discuss the therapeutic approaches and pathogenesis of this syndrome from deep intra-wall vascular calcification to the resulting infarctions of adjacent tissues.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/7. Penile calciphylaxis: analysis of risk factors and mortality.PURPOSE: Penile calciphylaxis is a rare condition resulting in infection and gangrene. Most cases are associated with systemic calciphylaxis. The pathophysiology, diagnosis and management of penile calciphylaxis as a distinct entity have received little attention. We reviewed the literature to increase understanding of this disease. MATERIALS AND methods: A retrospective review of the literature was performed after treating a case of penile calciphylaxis. Patient characteristics, presentation, serum chemistry studies, management and outcomes are reported. RESULTS: A total of 34 cases of penile calciphylaxis were identified in the literature including our patient. Average patient age was 58 years. All patients had end stage renal disease, and diabetes mellitus was a co-morbidity in 76%. Additional areas of gangrene beyond the genitalia were found in two-thirds of patients. Average calcium phosphate product was 78.5 mg.2/dl.2 (range 20.6 to 52.5) and mean parathormone level was 553 pg./ml. (10 to 65). parathyroidectomy was performed in 8 patients. All patients were treated with either local debridement/wound care or partial/total penectomy. survival was better in patients who underwent parathyroidectomy (75%) than in those treated with local debridement or penectomy alone (28%). The overall mortality associated with this disease was 64% with a mean time to death of 2.5 months. CONCLUSIONS: Penile calciphylaxis is a result of medial calcification and fibrosis of blood vessels. The co-morbidity and mortality associated with this disease are extremely high. Secondary hyperparathyroidism and an increased calcium phosphate are characteristic and require aggressive medical management. Surgical management of penile lesions and parathormone is controversial. Our review suggests that parathyroidectomy may improve survival and that survival is independent of the type of local treatment for the penile lesions.- - - - - - - - - - ranking = 5keywords = calciphylaxis (Clic here for more details about this article) |
3/7. 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.- - - - - - - - - - ranking = 3.5keywords = calciphylaxis (Clic here for more details about this article) |
4/7. nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes.nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.- - - - - - - - - - ranking = 4.5keywords = calciphylaxis (Clic here for more details about this article) |
5/7. Proximal calciphylaxis in four insulin-requiring diabetic hemodialysis patients.We present four cases of proximal calciphylaxis in end-stage renal disease patients treated with hemodialysis. All patients were diabetic and developed lesions in areas that had previously served as sites of insulin injection. We review the presentation, pathogenesis, pathology, prognosis, and treatment of this devastating condition. Finally, we hypothesize that subcutaneous injection of insulin may play a pathogenic role in the development of proximal calciphylaxis.- - - - - - - - - - ranking = 3keywords = calciphylaxis (Clic here for more details about this article) |
6/7. Cryofibrinogenemia: an addition to the differential diagnosis of calciphylaxis in end-stage renal disease.Cryofibrinogenemia is a disorder characterized by cryoprecipitation with variable clinical presentation that was first described by Korst and Kratochvil in 1955. Cryofibrinogen is a cold insoluble complex of fibrin, fibrinogen, and fibrin split products with albumin, cold insoluble globulin, factor viii, and plasma proteins. Cryofibrinogenemia is associated with metastatic malignancies, collagen vascular diseases, and thromboembolic disorders and may be clinically asymptomatic or present with thromboembolic phenomena of skin and viscera. The pathogenesis of cryofibrinogenemia is unknown. It may be caused by the inhibition of fibrinolysis, leading to an accumulation of cryofibrinogen. Treatment of cryofibrinogenemia may include stanozolol, plasmapheresis, and fibrinolytics. Cryofibrinogenemia simulates calciphylaxis clinicopathologically, because both may present with skin necrosis. calciphylaxis has been reported in end-stage renal disease, but we report the first case of cryofibrinogen in a chronic dialysis patient. We suggest that in the appropriate clinical setting, cryofibrinogenemia should be considered in the differential diagnosis of calciphylaxis, and serum cryofibrinogen levels should be measured in end-stage renal disease patients presenting with skin necrosis.- - - - - - - - - - ranking = 3keywords = calciphylaxis (Clic here for more details about this article) |
7/7. Breakdowns on the path of chronic illness: opportunities for learning.An unusual case of calciphylaxis, presenting at the onset of end-stage renal disease and evolving into an extended and arduous hospital stay, is described. The medical approach to this case is addressed briefly, but the main focus of this paper is to describe, in the words of various participants, the events and interactions that occurred and to learn from this description how our management of such cases breaks down. When confronted by difficult circumstances, it is common for us to react emotionally in ways that are automatic and based on our own personal histories and behavior patterns. Such automatic reactions prevent us from seeing and understanding what we really need to know about a given situation and leave us vulnerable to discouragement and internal suffering when clinical events do not go well (A. Nierenberg, personal communication, April 1998). The result is often exasperation with patients and families, as well as emotionally laden interactions that do not forward problem solving. In retrospect, the appearance of such breakdowns is not only predictable in the course of chronic illness, but offers us the opportunity to observe our automatic reactions, to re-evaluate our approach, and to redesign our actions. We have written this review, not to find error or blame, but rather to emphasize that we are learning to view these breakdowns as signals first to step back from our automatic reactions and then to listen and communicate clearly as a means to navigating the best pathway through difficult and discouraging clinical challenges.- - - - - - - - - - ranking = 0.5keywords = calciphylaxis (Clic here for more details about this article) |