1/18. nephrogenic fibrosing dermopathy with systemic involvement.BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/18. An unusual case of calciphylaxis.BACKGROUND: Cutaneous calciphylaxis is a rare disorder that occurs most frequently in patients with end-stage renal disease (ESRD), those on hemodialysis, and renal transplant recipients. It is frequently associated with hyperparathyroidism and a markedly elevated calcium-phosphate product, and it carries a high mortality rate. The usual clinical presentation is of painful, stellate necrosis of the thighs or buttocks, often in the setting of livedo reticularis. death usually results from septicemia. OBJECTIVE: This report documents an unusual case of recurrent, self-limiting calciphylaxis in the setting of a patient with ESRD and discusses the clinical and pathologic features of this potentially very fatal disorder. methods AND RESULTS: A 52-year-old woman presented with a greater than one-year history of relapsing and remitting, exquisitely painful, necrotic, numular plaques on the abdomen, breast, and arm. This patient had a markedly elevated calcium-phosphate product and parathyroid hormone level. The diagnosis of calciphylaxis was made by wedge biopsy of the most recent plaque, revealing calcification of medium-sized subcutaneous vessels and lobular capillaries with associated epidermal necrosis. CONCLUSIONS: This case demonstrates an unusual clinical variant of calciphylaxis that presented without the characteristic stellate necrosis or livedo reticularis that normally marks this condition and spontaneous resolution without incurring septicemia. Regardless of morphology, calciphylaxis should be considered in the differential diagnosis of painful, necrotic lesions occurring in the setting of ESRD.- - - - - - - - - - ranking = 9keywords = calciphylaxis (Clic here for more details about this article) |
3/18. calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathy.BACKGROUND: calciphylaxis and metastatic calcification are known complications of chronic renal failure. Recently, a sclerosing condition of the skin termed nephrogenic fibrosing dermopathy (NFD) has been described in patients with renal disease, many of whom have undergone hemodialysis and/or renal transplantation. To our knowledge, the simultaneous occurrence of both conditions in the same patient, in the same lesion, has not been previously reported. CASE REPORT: We report the clinical, microscopic, and immunohistochemical features of two patients with chronic renal failure whose lesional skin biopsies showed both subcutaneous calcification and NFD. We consider the possible mechanisms that might explain the coexistence of these two disorders. RESULTS: Both patients presented with erythematous, indurated skin over the lower extremities. Purpuric, reticulated patches, necrosis, or ulceration were not observed. Microscopic examination showed the characteristic changes of NFD involving dermis and subcutaneous septa. In addition, biopsies of both individuals showed subcutaneous calcification, one in a diffuse distribution and the other involving the walls of subcutaneous vessels, as seen in calciphylaxis. Calcification was not suspected clinically in either case. CONCLUSIONS: Metastatic calcification or calciphylaxis and NFD can occur simultaneously in patients with chronic renal failure and may be found together in the same lesion. Because subcutaneous calcification may not be suspected clinically in these cases, and in view of the adverse outcomes frequently associated with calciphylaxis, we recommend deep incisional biopsy of patients presenting with the clinical features of NFD. Both the fibrosis and the calcification of chronic renal failure may be related to the activity of transforming growth factor-beta/Smad signaling cascades.- - - - - - - - - - ranking = 3keywords = calciphylaxis (Clic here for more details about this article) |
4/18. calciphylaxis associated with acute, reversible renal failure in the setting of alcoholic cirrhosis.We describe a case of calciphylaxis in a 47-year-old man with alcohol-induced end-stage liver disease and acute renal failure secondary to hepatorenal syndrome. Possible contributing factors included transiently impaired renal function, protein c and S deficiencies, elevated calcium-phosphate product, hyperphosphatemia, low serum albumin, repeated albumin infusions, and elevated alkaline phosphatase level.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
5/18. Traumatic calcinosis cutis in a dialysis patient.A patient with end-stage renal disease and refractory hyperparathyroidism was evaluated for acute-onset thickening and hardening of the skin of the lower extremities. Her clinical course and physical examination findings were consistent with the recently described entity of nephrogenic fibrosing dermopathy. However, skin biopsy results showed metastatic and dystrophic calcification, without calcific uremic arteriolopathy (calciphylaxis). The patient reported a history of self-inflicted trauma; the authors postulate that trauma, in the setting of hyperparathyroidism and an elevated serum calcium phosphorous product, resulted in the subcutaneous deposition of calcium salts. To the authors' knowledge, this is the first report of metastatic and dystrophic calcification, without calciphylaxis, in a patient with refractory hyperparathyroidism. This case underscores both the rich variety of skin conditions seen in patients undergoing dialysis and recent developments in the field of dermatologic disorders associated with end-stage renal disease.- - - - - - - - - - ranking = 2keywords = calciphylaxis (Clic here for more details about this article) |
6/18. Forensic dermatopathology and internal disease.The gross and microscopic analysis of skin lesions at autopsy can help the pathologist understand diseases and injuries inflicted premortem, perimortem, or postmortem. From January 2003 to January 2004, skin findings at autopsy were closely examined by a dermatologist and sampled for microscopic analysis at the Southwestern Institute of forensic sciences. Dermatologic abnormalities in some of these cases led to the discovery of internal disease and allowed for a more complete understanding of the pathologic disease processes affecting the individual. We present four autopsy cases with skin manifestations of internal disease, including pseudoxanthoma elasticum, calciphylaxis, the sign of Leser Trelat, and papular mucinosis, and demonstrate the usefulness of the dermatological assessment at autopsy. In all cases, discovery of these skin lesions and internal disease manifestations allowed contributing factors to the death of the individual to be uncovered.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
7/18. calcinosis cutis universalis in a patient with systemic lupus erythematosus.Deposition of calcium salts in the skin and subcutaneous tissue occurs in a variety of rheumatic diseases, being most commonly associated with scleroderma, CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia), dermatomyositis, and overlap syndromes but is a rare complication of systemic lupus erythematosus (SLE). calcinosis is classified into four subsets: dystrophic, metastatic, idiopathic, or calciphylaxis/iatrogenic. The pathophysiology of calcinosis cutis remains unclear. Our patient developed extensive areas of calcifications in the trunk and extremities (calcinosis universalis) 8 years after SLE diagnosis, which would correspond to a form of dystrophic calcification. No response was observed after treatment with oral diltiazem for 3 months. We review the literature on the pathogenesis and prevalence of calcinosis universalis in SLE.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
8/18. Widespread cutaneous and systemic calcification (calciphylaxis) in patients with the acquired immunodeficiency syndrome and renal disease.BACKGROUND: calciphylaxis is a form of widespread calcification that may occur in patients with renal disease and hyperparathyroidism. The skin is often affected secondary to vascular compromise and ischemia. patients infected with the human immunodeficiency virus (hiv) are predisposed to renal failure and thus may develop this serious complication. OBJECTIVE: The purpose of this article is to describe two cases of fatal calciphylaxis in patients with the acquired immunodeficiency syndrome and renal disease. methods: Two patients were examined clinically and through the performance of serologic studies and skin biopsies. RESULTS: Both patients had a widespread livedo reticularis-like eruption, which rapidly progressed to cutaneous gangrene. Patient 1 had elevated serum calcium and phosphorus levels whereas patient 2 had normal values but had markedly elevated serum parathyroid hormone levels. biopsy specimens in both cases showed vascular thrombosis and soft tissue calcification. CONCLUSION: Widespread calcification may develop in patients with hiv infection and renal failure and, in some cases, calcium and phosphorus levels may be normal.- - - - - - - - - - ranking = 5keywords = calciphylaxis (Clic here for more details about this article) |
9/18. association between calciphylaxis and inflammation in two patients on chronic dialysis.The pathogenesis of calciphylaxis, which has a rising incidence in the chronic dialysis population and a high mortality rate, is poorly understood. Abnormalities in the calcium-phosphorus-parathyroid axis are clinically related to calciphylaxis, but alone, they cannot explain this condition. Here, we present two patients who had chronic inflammatory conditions and hyperparathyroidism and who developed calciphylaxis. A 41-year-old white woman on hemodialysis following scleroderma, hepatitis c, liver transplant, and failed kidney transplant, developed progressive ulcerative lower extremity calciphylaxis lasting more than 3 years. She had evidence of severe hyperparathyroidism and elevated serum c-reactive protein (CRP). A 39-year-old white woman on continuous ambulatory peritoneal dialysis for 6 years for renal failure secondary to lupus nephritis, with sustained lupus activity during the dialysis period, developed rapidly progressing ulcerative calciphylaxis of the lower and upper extremities not responding to adequate treatment of hyperphosphatemia and hyperparathyroidism. Her condition culminated in death within 2 months of the appearance of the skin lesions. Her serum CRP was elevated on a sustained basis before the development of the calciphylaxis and rose to a very high level after appearance of the skin lesions. inflammation may assist in the development of calciphylaxis through depression of serum levels of fetuin-A, an endogenous inhibitor of calcification that is also a negative acute-phase reactant. The interactions between inflammation-mediated changes in the levels of endogenous inhibitors of calcification and abnormalities in calcium-phosphorus metabolism merit intensive study in the future as potential mechanisms of calciphylaxis.- - - - - - - - - - ranking = 12keywords = calciphylaxis (Clic here for more details about this article) |
10/18. 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 = 9keywords = calciphylaxis (Clic here for more details about this article) |
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