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Calciphylaxis is a rare disease that is caused by calcium deposition in medium and small sized vessels of the skin causing ischemic necrosis. These lesions often become infected and cause septicemia. We report a unique case of penile and scrotal calciphylaxis that rapidly progressed to Fournier's gangrene. We review the etiology of calciphylaxis and also its management.
Calciphylaxis is a rare and potentially fatal complication of end stage renal disease (ESRD) and secondary hyperparathyroidism associated with abnormal calcium metabolism. Painful purple skin lesions are often the presenting sign with digital ischemia, ulcerations, and necrosis being the characteristic features of the disease. Skin or incisional biopsy showing widespread calcifications and fibrinous thrombi without inflammation will confirm the diagnosis. Early recognition and prompt treatment of calciphylaxis is vital for symptom relief, ulcer healing, and prevention of sepsis and death. This article will educate nurses to recognize this disease in hope of early treatment. The article includes information about disease history, pathogenesis, etiology, clinical manifestations, diagnosis, prevention, and current treatment, and, in addition, presents case study of a patient with calciphylaxis.
Tissue calcification is a well-recognized common metabolic disease, but calciphylaxis still remains an enigmatic rarity. The latter may be induced experimentally and acquired naturally in human diseases. Although many chronic azotemic or end stage renal disease patients (ESRD) with hyperparathyroidism (HPT) are at risk of calciphylaxis not all of them do develop the disease, even non-renal, patients may also develop this disease. Out of a total of about 2000 hemodialysis, 15,000 dermatology and 26,000 medical patients seen over a three year period in a busy Saudi Arabian tertiary medical centre, we report a sentinel nephrology patient with sudden excruciatingly painful cutaneous calciphylaxis that necessitated acute dermatology emergency consultation, and present a review of the medical literature. In order to institute appropriate total quality management of this life-threatening, rare disease, it is advisable that a high index of suspicion should be entertained by dermatologists, general physicians, nephrologists, and pulmonologists in an appropriate clinical scenario.
Calciphylaxis is a rare disorder in patients with chronic renal failure that is characterized by ischemic necrotic skin lesions. The prognosis is grave and mortality is high (80%). The precise mechanism of calciphylaxis is still unknown, but in addition to chronic renal failure, elevated parathyroid hormone levels appear to play a role. The role of parathyroidectomy in treating affected patients is questionable. In this article, we describe the case of a patient with chronic renal failure who developed rapidly progressive subcutaneous calcifications and ulcerations in the lower extremities. These lesions regressed following subtotal parathyroidectomy. We also review the literature on calciphylaxis, with a focus on treatment options.
An unusual protracted course of calciphylaxis without cutaneous ulcerations has been encountered in 14 cases. Calciphylaxis may include several clinical presentations ranging from an acute, rapidly fatal course to an indolent, more benign variant. Calciphylaxis should be included in the differential diagnosis for patients with kidney disease presenting with a clinical picture of panniculitis. Patients with renal disease with a characteristic clinical presentation with or without cutaneous ulcerations should undergo an urgent biopsy to confirm the diagnosis.
The problem of abacterial nephropathies associated with impaired calcium metabolism is discussed from the standpoint of membranous pathology. The data are presented on the similarity of the calciphylaxis pathogenesis described for the first time by H. Selye to calcium nephrolithiasis. The disease is viewed as the result of pronounced modification of the phospholipid layer of cell membranes under the influence of peroxidation, phospholipase activity and other processes induced by calcium-regulating hormones (parathyroid hormone, D3 and calcitonin). It is assumed that the clinical manifestations of renal pathology during calciphylaxis depend on the hereditary and environmental factors that determine the gravity of membranous pathology of renal cells. Recommendations as to the treatment and prevention of renal pathology during calciphylaxis based on the results of the authors' own experience are given.
BACKGROUND: Cutaneous necrosis of the proximal lower extremities in a patient with end-stage renal disease is the classic presentation of calciphylaxis, an untreatable, rare, generally fatal necrotizing cutaneous syndrome. Type 1 primary hyperoxaluria (PH-1) usually presents in childhood with recurrent urolithiasis. Since enzymatic studies to confirm the metabolic defect are now available, some cases of idiopathic renal failure in adulthood have been shown to be caused by PH-1. These patients may develop vascular oxalate deposits resulting in livedo reticularis and distal acral vascular insufficiency. OBSERVATIONS: We describe a patient who presented in end-stage renal failure with proximal lower extremity cutaneous necrosis suggestive of calciphylaxis. A cutaneous biopsy specimen revealed oxalate crystals within blood vessels, and a diagnosis of PH-1 was confirmed enzymatically. CONCLUSIONS: This patient illustrates that PH-1 may present in adulthood, and, in the setting of cutaneous necrosis associated with end-stage renal disease, it may be confused with calciphylaxis. The importance of making a diagnosis of PH-1 is the potential ability to achieve long-term survival by reversing the underlying metabolic defect with hepatic transplantation.
A 31-year-old woman with systemic lupus erythematosus (SLE), diabetes mellitus, and chronic renal failure developed digital ischemia, myocardial dysfunction, abnormal ECG, and elevated CPK levels. Radiographic studies revealed calcification of the peripheral vasculature although coronary angiography was unremarkable. An endomyocardial biopsy demonstrated intra and extracellular myocardial calcification without evidence of vasculitis or primary myocarditis. A diagnosis of calciphylaxis, as a result of secondary hyperparathyroidism, was made. This case demonstrates that calciphylaxis can mimic the cardiovascular manifestations of SLE. Early differentiation of these disparate diseases is important because treatment strategies employed in SLE may exacerbate calciphylaxis.
A 46-year-old male patient underwent long-term hemodialysis treatment had suffered from calciphylaxis (defined by Selye), such symptoms as advanced systemic vascular calcification, rapid progression of gangrene on both fingers and toes, disturbance of consciousness, and sclerosis and obstruction of the superficial vein after venipuncture during 11.5 years of dialysis. Furthermore, he had a long history (30 years) of heavy smoking. He died as a result of sepsis due to pneumonia after 12.5 years of dialysis. He had received dialysis treatment using a small amount of dialysate (50 liters on a recirculating system) for 8.5 years and had been dialysed 2 and 2 or 3 times a week for 10 years. As a result of this insufficient dialysis treatment, his characteristic laboratory data showed hypocalcemia, hyperphosphatemia, elevated calcium-phosphorus product, advanced metabolic acidosis, hyperalkaliphosphatemia and elevated serum parathyroid hormone. Autopsy revealed the following: 1) enlargement parathyroid gland enlarged in two (4.0 g and 2.0 g, respectively) showing adenomatous hyperplasia presenting cord-like arrangement of chief cells and water-clear cells, 2) systemic medial calcification in radial, ulnar, renal, mesenteric and brain arteries, and 3) Berline-blue positive iron deposit in calcified arteries in mesenteric and parathyroid tissue. From these results, we concluded that factors (challengers) related to the appearance of calciphylaxis might be as follows: 1) advanced secondary hyperparathyroidism, 2) long-term uremic state, 3) administration of VD2 and VD3, 4) iron salt injection, and 5) a long history of heavy smoking. We speculated that these challengers might act synergistically to cause calciphylaxis.
Three cases of calciphylaxis in patients with terminal renal insufficiency are discussed. The existence of metastatic calcifications, mainly vascular, in patients with chronic renal insufficiency is frequent. However calciphylaxis is a process which is rarely found in these patients. This entity is characterized by the obliteration of small vessels with ischemia and necrosis. The exceptionality of the disease, its difficult treatment and poor evolution make calciphylaxis a bad prognostic complication which should be considered in the differential diagnosis of cutaneous lesions in patients with chronic renal insufficiency.
A 38-year-old woman with insulin-dependent diabetes mellitus, hypertension, and end-stage renal disease developed digital ischemia, widespread cutaneous necrosis and eschar formation of both lower extremities, and extensive ulceration of the large intestine and cecum resulting in gastrointestinal hemorrhage. A mesenteric arteriogram revealed multiple stenotic areas and filling defects of the superior mesenteric artery and its tributaries, suggestive of vasculitis. A diagnosis of calciphylaxis was suspected, on antemortem skin biopsy, and was later confirmed by postmortem examination. This case further documents the relationship between calciphylaxis and significant visceral injury, and it represents, to our knowledge, the first case of calciphylaxis associated with massive gastrointestinal hemorrhage.
Several types of soft tissue calcification can be detected from radiographic evaluation of patients with end-stage renal failure. The factors that predispose to such calcification include an increase in CaxP product in serum, the degree of secondary hyperparathyroidism, the level of blood magnesium, the degree of alkalosis, and the presence of local tissue injury. Three major varieties include calcification of medium-sized arteries, periarticular or tumoral calcification and visceral calcification. Calciphylaxis is a phenomenon consisting of acute ischemic necrosis in presence of calcification of dermohypodermic arterioles. It occurs mostly in chronic renal failure patients with secondary or tertiary hyperparathyroidism with a persistently elevated calcium-phosphorus product. There are few options in treating calciphylaxis and the outcome is generally poor. The authors report the case of a haemodialised patient with benign nodular calcification and calciphylaxis. The coexistence of both entities in the same patient has never been described.
Two male patients with chronic renal failure maintained on hemodialysis developed progressive clinical features of ischemic necrosis (so called calciphylaxis) of their extremities and penis. Both patients died. In one patient, penectomy provided tissue for histopathologic examination and there were changes of small artery calcification. A role of iron overload on the production of calciphylaxis is reviewed. This report is perhaps the first in nephrology literature on the occurrence of calciphylaxis involving penis and prepuce.
A 43-year-old woman was admitted with progressive leg pains and weakness and was found to have rhabdomyolysis. Prior to this admission the patient had hypercalcemia, but this returned to normal following treatment with calcitonin. During the hospitalization, she developed the syndrome of calciphylaxis consisting of necrotic skin and muscle associated with vascular calcification. This is the first case report of rhabdomyolysis caused by calciphylaxis in a patient without chronic renal failure.
Systemic calcification syndromes are a recognized complication occurring in some patients with end-stage renal disease (ESRD) and secondary hyperparathyroidism. These patients develop severe livedo reticularis and subcutaneous tissue lesions progressing to frank necrosis and ultimately large areas of eschar. Clinically this syndrome is known as calciphylaxis; these lesions are usually resistant to aggressive debridement, systemic antibiotics, and revascularization procedures. We report three patients with somewhat different clinical presentations but all sharing a common link of exquisitely painful leg ulcers initially being treated as ischemic lesions or venous stasis-type ulcerations. These three patients were diagnosed with calciphylaxis on the basis of clinical, biochemical, and histopathological criteria. Two patients underwent parathyroidectomy late in the progression of their disease, with some resolution of their ulcerative lesions.
Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification that is seen almost exclusively in patients with end-stage renal disease and hyperparathyroidism. We report a case of calciphylaxis that may very well be due to a long-term steroid use in a rheumatoid arthritis patient who had neither end-stage renal disease nor hyperparathyroidism. We also discuss the possible role of protein S deficiency as a contributing factor.
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.
A 58-year-old white woman on continuous ambulatory peritoneal dialysis for 2 years developed calciphylaxis-induced necrotic skin lesions over both lower extremities. Despite subtotal parathyroidectomy and other conventional measures, skin lesions continued to worsen. Mapping of transcutaneous oxygen pressure showed markedly low values in involved areas. Skin ulcers completely healed after 38 sessions of hyperbaric oxygen therapy. The results in our case indicate that hyperbaric oxygen therapy may be useful in the treatment of skin ulcers secondary to calciphylaxis.