Calciphylaxis: what nurses need to know. (Continuing Education).
Calciphylaxis, as defined in the literature, is a uremic small artery disease with widespread calcification of small and intermediate vessels (McAuley, Devereux, & Walker, 1997). Other names that have been used to describe calciphylaxis include uremic gangrene syndrome (Edwards, Jaffe, Arrowsmith, & Henderson, 2000), metastatic calcinosis, azotemic calcific arteriopathy, and intimal hyperplasia, but the most functional term may be the more recent vascular calcification-cutaneous necrosis syndrome (Flanigan et al., 1998). Systemic calciphylaxis is an entity consisting of progressive, severe, ischemic necrosis encompassing widespread anatomical regions.
This disease entity, that will continue to be referred to as calciphylaxis in this article, is an uncommon condition that is poorly understood. This article will educate nurses about calciphylaxis, so that they can help in detecting the signs and symptoms of this disease and implement and evaluate treatment.
Morbidity and Mortality
Calciphylaxis is a rare phenomenon that presents in less than 1% of ESRD patients each year, including transplanted patients (Essary & Wick, 2000; Sankarasubbaiyan, Scott, & Holley, 1998). By 1998, only a total of 155 patients had been described in the literature (Edwards et al., 2000). The incidence of this syndrome is higher among younger patients who have had hemodialysis for longer periods of time than the typical hemodialysis population. The prevalence rate of calciphylaxis in one reported hemodialysis unit was 4.1% (Angelis, Wong, Myers, & Wong, 1997). This disease is almost exclusively seen in ESRD patients (McAuley et al., 1997).
There is a great deal of morbidity and mortality from calciphylaxis, largely caused by nonhealing ulcers and secondary infections leading to uncontrolled sepsis and oftentimes death (Green, Green, & Minott, 2000; Oh, Eulau, Tokugawa, McGuire, & Kohler, 1999). Mortality is over 60% for patients with leg ulcers. Patients suffering from proximal calciphylaxis (occurring predominantly on the abdomen and thighs) have a much worse prognosis (Bleyer, Choi, Igwemezie, de la Torre, & White, 1998). The overall mortality rates of calciphylaxis have ranged from as low as 60% (Green et al., 2000; Randall et al., 2000) to as high as 80% (Green et al., 2000), with patients progressing to death within a relatively short time after initial presentation (Essary & Wick, 2000). Early recognition may direct treatment and lessen the morbidity and mortality of this disease (Oh et al., 1999).
Calciphylaxis has been reported in CKD patients since as early as 1898 (Bleyer et al., 1998). Very little was known or researched about this syndrome, however, until Hans Selye began animal experimentation (Edwards et al., 2000; Handa & Strzelczak, 1998) originally to explain an experimental phenomenon in rats, which he thought was secondary to hypersensitivity and similar to anaphylaxis (Flanigan et al., 1998; Selye, 1962).
Selye administered vitamin D, parathyroid hormone (PTH), and calcium salts and induced renal failure in rats as conditioning factors that predisposed or "sensitized" rats to soft tissue calcification (Handa & Strzelczak, 1998; Selye, 1962). After a latent period, known as the "critical period," the rats were exposed to "challenging agents," such as local trauma (pinching skin or plucking hair) or intravenous infusion of compounds including egg albumin, iron salts, mast cell degranulators, and polymyxin (Angelis et al., 1997; Handa & Strzelczak, 1998; Selye, 1962). The phenomenon that resulted consisted of soft-tissue calcification, cutaneous necrosis, and inflammation and calcinosis in nearly all organs of the body (Angelis et al., 1997; Oh et al., 1999). Neither the sensitizer nor challenger alone in the concentrations used could induce this response (Oh et al., 1999). This led Selye to conclude that calciphylaxis is a condition of induced hypersensitivity in which tissues react to challenging agents with calcium deposition (Handa & Strzelczak, 1998). Selye's theory, although almost 40 years old, remains undisputed.
Pathogenesis and Etiology
The pathogenesis of calciphylaxis is uncertain (Angelis et al., 1997). It is unknown whether a similar sequence of events as depicted was with the rats, occurs in what we see today in human calciphylaxis (Handa & Strzelczak, 1998). In relating Selye's work to human calciphylaxis, it could be conjectured that ESRD, secondary hyperparathyroidism, hyperphosphatemia, and a high calcium-phosphate product may serve as components of the sensitizing arm. Various medications, local trauma (from subcutaneous injections), or abnormalities in blood constituents could be a branch of the "challenging" arm (Essary & Wick, 2000). The medications suggested in the literature to be challengers include immunosuppressants, such as corticosteroids, cytotoxic drugs (azathioprine) (James et al., 1999), and vitamin D (Flanigan et al., 1988). A pre-existing hypercoagulable state has also been suggested to predispose affected patients to calciphylaxis (Oh et al., 1999). Past studies, in particular, have theorized that a functional protein C abnormality or protein S deficiency could be likely causes of thrombotic events, representing the cause of ischemia in clients who are susceptible to developing calciphylaxis (Essary & Wick, 2000).
The etiology of calciphylaxis is not clearly defined in the literature. Calcium phosphate metabolism alteration is the most prominent characteristic of calciphylaxis (Bleyer et al., 1998) (see Figure 1). There is significant hyperphosphatemia combined with longstanding inadequately controlled renal failure and secondary hyperparathyroidism in the majority of cases. Secondary hyperparathyroidism may contribute to hyperphosphatemia by enhancing the release of phosphate and calcium from the bone (McAuley et al., 1997). The combination of hyperphosphatemia and low normal plasma calcium will result in an increased calcium phosphate product and the tendency to cause calcification of the medial layer of the small and medium-size vessels leading to tissue ischemia, necrosis, and gangrene. Hypoalbuminemia further augments influx of ionized calcium from a dialysate into extracellular fluid and results in precipitation of calcium into the soft tissue (Handa & Strzelczak, 1998). Soft tissue calcium deposition primarily affects the trunk or lower extremities causing infarction of the skin and underlying structures (Green et al., 2000). All dialysis patients suffer from disordered calcium metabolism, although very few patients develop calciphylaxis. For that reason, calciphylaxis is unquestionably multifactorial in etiology.
[FIGURE 1 OMITTED]
Hyperphosphatemia and an elevated serum calcium phosphate product may have a fundamental role during extended periods of maintenance hemodialysis leading to the pathogenesis of calciphylaxis (see Figure 1). Nonadherence to phosphate-binding therapy and failure to follow a low phosphate diet in these younger patients may also be implicated in this condition. Calciphylaxis appears to be more frequent in younger dialysis patients, perhaps as a consequence of long periods on dialysis (Oh et al., 1999) or different etiologies of renal failure (Angelis et al., 1997). A recently described etiologic theory is that infection by certain microorganisms may provide the impetus for pathologic calcification (Oh et al., 1999)
There are two classifications of calciphylaxis referred to in the literature: distal and proximal calciphylaxis. Distal (acral) calciphylaxis is described as lesions restricted to the regions distal to the elbows or knees (Bleyer et al., 1998), whereas proximal calciphylaxis can be described as lesions leading to gangrene occurring in the breast, thigh, abdomen, shoulder, and buttock region (Goldsmith, 1997). Diabetes may slightly raise the risk of distal gangrene (Oh et al.,1999); however, characteristically, diabetics are more prone to have proximal calciphylaxis (Handa & Strzelczak, 1998).
There may be an association between hypertension and calciphylaxis, because hypertension along with diabetes causes small and medium vessel pathology, which may exacerbate or accelerate calciphylaxis (McAuley et al., 1997). Other conditions with less common association with calciphylaxis include multiple myeloma, lymphoma, metastatic breast cancer, leukemia, systemic lupus erythematous, Crohn's disease, and AIDS (Essary & Wick, 2000).
Possible documented risk factors of calciphylaxis include poor nutritional status (Bleyer et al., 1998), obesity (determined by high body mass index), low serum albumin (Handa & Strzelczak, 1998; James et al., 1999), and White race (James et al., 1999). Pressure in the skin folds perhaps could be a potentiating factor as could skin temperature or shear injury (Oh et al., 1999). There is currently no reliable way to identify the typical patient who is at risk for developing this disease.
The key features of systemic calciphylaxis are digital ischemia and widespread cutaneous and muscular necrosis (Brown, Denney, & Burns, 1998). Skin lesions are often the presenting sign (Flanigan et al., 1998). Calciphylaxis generally manifests with tender, violet-colored or erythematous, mottled skin lesions, known as livedo reticularis (Angelis et al.,1997; Essary & Wick, 2000). These lesions usually progress rapidly over several days to weeks to painful indurated plaques or nodules in the skin and subcutaneous tissues (Goldsmith, 1997), resulting in ulcers and thick dark eschars with underlying tissue necrosis associated with considerable pain (Essary & Wick, 2000). The lesions are usually limited to the distal extremities with digital necrosis and gangrene being the foremost findings. However, ischemic necrosis may occur in the abdomen, thigh, breasts, and buttocks. Lesions present on the limbs and trunk and can be large in size (Edwards et al., 2000). Lesions appear frequently in the crease of the lower abdomen or on the medial aspect of both thighs in a "kissing" manner (Bleyer et al., 1998). Involvement of the penis has also occurred (Oh et al., 1999).
These lesions of proximal calciphylaxis are typically complicated with calcifications of subcutaneous fat and secondary infections (Goldsmith, 1997). Lesions frequently do not heal, but if they do heal, it is an exceedingly slow process (Bleyer et al., 1998). With time, potential complications often occur including digital gangrene necessitating amputation, pancreatitis, sepsis, multi system organ failure, and death (Angelis et al., 1997). It has been reported by Ivker, Woosley, & Briggaman (1995) that calciphylaxis may cause manifestations of ischemic damage to myocardium, brain, and intestine along with dementia, fever, and hypotension. Other calciphylaxis manifestations have included calcification of the lungs, kidneys, adrenals, and stomach in the setting of hyperparathyroidism (Khafif, DeLima, Silverberg, & Frankel. 1990).
The diagnosis of calciphylaxis can be inferred by clinical history and cutaneous examination (Oh et al., 1999). However, an incision biopsy will verify the diagnosis (Edwards et al., 2000; Oh et al., 1999). Goldsmith (1997) advocates skin biopsy even with the risk of poor healing in order to confirm calciphylaxis as the disease entity in all cases so that rational decisions can be made regarding disease management. A skin biopsy that illustrates widespread calcification and fibrinous thrombi occluding vessel lumina plus the absence of inflammation clinches the diagnosis (Asirvatham, Sebastian, Sivaram, Kaufman, & Chandrasekaran, 1998; Goldsmith, 1997; Handa & Strzelczak, 1998; McAuley et al., 1997). Calcium deposits in the walls of small to intermediate diameter blood vessels of the dermis and subcutaneous fat precedes fibroblast proliferation and giant cell function. Fibrosis, intimal hyperplasia, and occasional thrombosis of the vessels occur. Ischemia and necrosis of the skin ultimately result, which leads to the gross clinical observations in calciphylaxis (Oh et al., 1999). There are no diagnostic laboratory tests for calciphylaxis. Radiographs usually show a fine double-lined network of calcification of the arteries (Asirvatham et al., 1998) but are not diagnostic.
The diagnosis of calciphylaxis is not always obvious. Diabetic ulcers do not usually cause pain. Peripheral vascular disease causes nonhealing, painful ulcers of the lower extremities in renal failure patients. Calciphylaxis is the probable diagnosis if peripheral pulses are intact, proximal ulcers are present, and there is no evident neuropathy. If painful gangrene occurs in uremic patients (Angelis et al., 1997), calciphylaxis should be considered.
The clinical differential diagnosis of the skin lesions is that of livedo reticularis and purpura (Oh et al., 1999). The variability of skin lesions, however, may lead to diagnoses that resemble calciphylaxis, including disseminated intravascular coagulation, warfarin necrosis, cryoglobulinemia, cryofibrinogenemia, pyoderma gangrenosum, lipodermatosclerosis, connective tissue disease (Randall et al., 2000), vasculitis (Wegener's granulomatosis) (Flanigan et al., 1998), antiphospholipid syndrome, cellulitis (Bleyer et al., 1998), diabetic ulcers (Angelis et al., 1997), diabetic muscle infarction (Goldsmith, 1997), deep fungal infections, dermatomyositis, necrotizing fasciitis, metastatic calcinosis, pseudoxanthoma elasticum (Oh et al., 1999), oxalosis, atherosclerotic peripheral vascular disease, embolic phenomena (atheroembolization or septic embolism), and isolated acquired protein S or C abnormalities (Essary & Wick, 2000) (see Table 1). The differential diagnosis of calciphylaxis should always include diabetes, microembolization, peripheral vascular disease, and autoimmune vasculitis (Edwards et al., 2000).
Prevention and Treatment
The most important strategy for preventing calciphylaxis is early detection of renal failure, aggressive control of urea, and control of calcium and phosphorus with the suppression of secondary hyperparathyroidism (McAuley et al., 1997). Targeting at-risk, morbidly obese, White, renal failure patients and those with poor nutritional status should be done, since these have been reported as risk factors. These patients need a special focus given to nutritional status along with maintaining a normal systolic blood pressure to sustain good tissue perfusion (Bleyer et al., 1998).
Early diagnosis of calciphylaxis is critical because it allows prompt treatment and possibly alters the grave prognosis associated with this disease. The treatment of choice for calciphylaxis is parathyroidectomy. PTH has been demonstrated to be a sensitizer in experimental calciphylaxis (Flanigan et al., 1998). Some patients have shown marked improvement after parathyroidectomy (Bleyer et al., 1998; Edwards et al., 2000; James et al., 1999; Lipsker, Chosidow, Martinez, Challier, & Frances, 1997). Prompt parathyroidectomy can bring about rapid relief of symptoms and wound healing, which may be limb and lifesaving (Edwards et al.,2000), but frequently the prognosis is still poor (Green et al., 2000). The role of parathyroidectomy is vague in cases in which PTH levels are not elevated (Sankarasubbaiyan et al., 1998). Early recognition of calciphylaxis allows the avoidance/removal of potential sensitizing and challenging agents, such as corticosteroids and immuno-suppressants, which should be discontinued even in renal transplant patients. Calciphylaxis has a much higher mortality than does the risk of renal graft failure (Randall et al., 2000).
The principal focus in treating calciphylaxis is reversing the underlying disease process and normalizing abnormal skin phosphorus and calcium levels. Aggressive control of phosphorus and calcium is mandatory (Sankarasubbaiyan et al., 1998). The use of low-calcium dialysate, oral phosphate binders, such as aluminum hydroxide gel, and low phosphate diet decrease the calcium-phosphorus product (Green et al., 2000; Lipsker et al., 1997). Calcium carbonate should be avoided due to a reported association with calciphylaxis (Flanigan et al., 1998). Early treatment of this disease should consist of local wound care and antibiotics to treat secondary infections. To prevent skin ulcers, wound infections should be treated with local wound debridement of necrotic tissue, incision and drainage of abscesses, and administration of systemic antibiotics, making sure to cover gram negative bacteria (Angelis et al., 1997).
Additional treatments that have reportedly met with some success include hyperbaric oxygen therapy (Essary & Wick, 2000; Sankarasubbaiyan et al., 1998), bone reabsorption inhibitors (Randall et al., 2000), and H2 histamine-blocking medications (Essary & Wick, 2000). There is debate on the use of exogenous corticosteroids. As described by Goldsmith (1997), steroids have little or no benefit in calciphylaxis and may even provoke sepsis, which is frequently the cause of death.
Implications for Nursing
Nurses should be familiar with this disease and assist in recognizing the signs so that treatment may be initiated earlier. All physical assessments in the renal failure population should involve a thorough exam of the skin, as well as questioning patients if they have noticed any skin changes, lesions, or tender areas on their bodies. Awareness of calciphylaxis, this rare entity occurring in ESRD patients with hyperparathyroidism, could save lives (Edwards et al., 2000).
Assessing and providing pain relief for patients is a major nursing responsibility that is oftentimes neglected in the hectic world nurses function in today. The majority of patients with calciphylaxis experience a significant amount of pain, which is not necessary. It is vital to treat their pain in a timely manner with appropriate medication to provide a reasonable quality of life. Nurses should use a consistent pain measurement tool to evaluate patient responses to interventions aimed at reducing pain.
Nurses also play a crucial role in wound management. They are routinely the clinicians who perform the dressing changes, evaluate the wound for infection and dissemination, and report the findings. Nurses perform a key role in teaching dressing changes to patients and their families. They also need to help patients with positioning to maximize comfort and relieve pressure to the ulcerated tissues.
Nurses can play a pivotal role in conducting and carrying out research on ESRD patients and calciphylaxis. There are many unanswered questions about disease prevalence, etiology, challenging and sensitizing agents associated with the disease, risk factors linked to it, and treatment. Tracking of calciphylaxis through dialysis registries may continue to reveal more answers about this rare disease. Unfortunately, there is not much research available on calciphylaxis since it is indeed an uncommon phenomenon. This places limitations on the development of large studies, and a large amount of our present information is based on case-controlled studies and case reports (James et al., 1999).
Finally, since nurses are the caregivers most frequently in contact with these patients during their state of pain, suffering, and possibly dying, they should be supportive of patients in their time of uncertainty. They can assist in arranging home health care and/or hospice if needed. Most importantly, they must maintain patient dignity and remember that there is a frightened person under that horrific disease.
A 57-year-old, African-American woman receiving hemodialysis for an 11-year history of ESRD secondary to hypertension was admitted to the nephrology service. She had an extensive past medical history, most notable diabetes mellitus type 2, peripheral vascular disease, parathyroidectomy 1 year ago, multiple episodes of peritonitis during prior peritoneal dialysis, diverticulitis, colectomy with transverse colostomy, gastric ulcer perforation, anemia, seizures, past smoker, and previous ETOH abuse. This patient was diagnosed with calciphylaxis 8 months prior to presenting to the emergency room with severe pain in her left lower extremity. On this admission, she was known to have a recent history of multiple infected ulcers and dry gangrene of her left lower extremity. This was her fourth admission for severe pain of lower extremity ulcers in 4 months. She did not have fever, chills, nausea, or vomiting on admission.
Physical exam revealed a tearful woman in moderate distress from severe leg pain. Her blood pressure was 132/75 mmHG, pulse was 126 beats per minute, and respiratory rate was 18. Neurologic and pulmonary exams were unremarkable. Cardiovascular exam revealed a grade II/VI systolic ejection murmur. The patient did have truncal obesity. The left lower extremity from the distal femur down showed areas of necrosis. Erythema and edema were present throughout the lower extremity. Nonhealing ulcers noted upon previous admissions from the past 3 months extended from the midcalf distally, encompassing 1/2 of the circumference of the leg. There were multiple new ulcers over the upper portion of the tibia. Serous weeping and purulent discharge was noted around blackened sections of her left foot. Pulses were 1+ in left lower extremity but difficult to obtain due to intermittent areas of intact skin. Large areas on the foot and lower extremity were denuded down to the fascia. Popliteal pulses were 2+ bilaterally. Dry gangrene of the left lateral 3 digits on the foot was present. Within 3 weeks of this admission, these lesions progressed from the left lower extremity to the buttocks, abdomen, and breasts bilaterally, starting over the areolae.
Peak laboratory results during this admission revealed a WBC 20.8/uL, blood urea nitrogen 32 mg/dL, creatinine 6.2 mg/dL, calcium 10.3 mg/dL, phosphorus 5.8 mg/dL, and protein C and S levels were both low at 71 and 43, respectively.
The patient was immediately started on IV antibiotics, ciprofloxacin and piperacillin/tazobactam, for infected ulcers. Blood cultures that were performed grew coagulase negative staphylococcus, for which she was appropriately treated. A chest x-ray performed several days after admission showed marked pulmonary edema with calcified subcarinal lymph nodes evident. High-grade (advanced) avascular necrosis was present in the right shoulder. A vascular report of the lower extremities showed bilateral ankle brachial index falsely elevated due to calcification.
Prior to this admission the patient had undergone many treatments for calciphylaxis including whirlpool therapy for 17 minutes a day, debridement of necrotic tissue, and dressing changes using silvadene/regranax. Prior to being diagnosed with calciphylaxis, the patient had already had a parathyroidectomy for other reasons, which is the treatment of choice for calciphylaxis. However, even with treatment, the lesions of calciphylaxis continued to spread. She received morphine sulfate and oxycodone/ acetaminophen for pain relief while in the hospital.
The patient underwent left above-the-knee amputation 7 days into this hospital admission for nonhealing ulcers and severe pain. The surgical pathology of the left above-the-knee amputation showed multifocal ulcerations and gangrenous necrosis with marked acute inflammation and granulation tissue. Atherosclerotic vascular disease was present. Fat necrosis was present at the soft tissue margin of resection. There were multiple ulcers on the anterior aspect of the leg and dorsal areas of the foot ranging in size from 1 x 1 cm to 15 x 8 cm. The ulcers did not appear to involve underlying bone. The skin of the toes and ventral surfaces of the foot showed black gangrenous discoloration. The skin and soft tissue at surgical margin appeared viable. Sectioning of the arteries revealed narrowed, but patent vessels.
The patient was started on gabapentin, an antidepressant, and was placed on an air mattress for pressure reduction. She was started on aggressive physical rehabilitation. During the tenth day of her hospitalization, the patient had mental status changes and slurred speech. The head CT scan showed mild cerebral and cerebellar atrophy, but no intracranial bleed was found. There was possible left inferior cerebellar focus of decreased density, probably representing small remote ischemic event. There was diffuse increased density of the calvarium, likely secondary to ESRD.
The patient died within 8 months of being diagnosed with calciphylaxis, with rapid progression of her lesions in the last 2 months prior to death. She had been transferred to hospice care 2 weeks prior to her death and treated with multiple pain medications in effort to promote comfort.
In summary, systemic calciphylaxis is a rare, progressive, life-threatening complication of ESRD and secondary hyperparathyroidism. It is characterized by diffuse ischemic necrosis. Treatment of this disease is always difficult, as described in the case study. Unfortunately, the patient in this scenario had already undergone the treatment of choice, parathyroidectomy, prior to her diagnosis of calciphylaxis. This case study does, however, show how vital early recognition truly is to reverse this potentially fatal disease, if even possible. Early diagnosis, appropriate medical management, diet therapy, wound care, and pain control, with parathyroidectomy, may slow or stop the disease progression, reduce limb loss, and prevent sepsis and death. Nurses can really improve the outcome of patients suffering from calciphylaxis by early recognition and implementing timely treatment of this life-threatening disease.
Table 1. Differential Diagnosis for Calciphylaxis Disease/Condition Symptoms and Findings Dermatomyositosis Fever, malaise, general weakness, erythematous/violaceous skin lesions without necrosis, elevated CK level, myopathy, and rare skin calcifications. Warfarin necrosis Necrosis of skin and soft tissue; painful, ecchymotic lesions leading to hemmorrhagic blisters that rupture and form new lesions. Antiphospholipid syndrome Features of stroke or TIA, pregnancy loss, distal leg ischemia or necrosis, superficial thrombophlebitis, blue toe syndrome, splinter hemorrhage, porcelain white scars, and positive antiphospholipid antibody. Diabetic muscle infraction Painful mass or swelling in calf; no skin lesions. CT scan, ultrasound, or MRI show enlargement of affected muscle. Histology shows focal muscle necrosis, leukocyte infiltration, intersitial fibrosis; and vessel occlusion by fibrin and calcium. Diabetic ulcers Nonhealing ulcers that are not painful. May have associated neuropathy and absent pulses. Ulcers are usually covered by calluses or fibrotic tissue. Disseminated intravascular Spontaneous bleeding, petechiae, purpura, coagulation and ecchymosis. Prolonged prothrombin time, partial thrombin time; decreased fibrinogen and platelet counts, increased fibrin degradation products, and decreased levels of factors II, V, VIII, and X. Vasculitis Organ and tissue ischemia, fevers, night sweats, weight loss, arthralgias, myalgias, and arthritis. Protein C and S deficiency Pain, tenderness, redness, or swelling in affected extremity and an increased tendency for intravascular blood clot formation. Cellulitis Infection of soft tissue, commonly in lower extremity, beginning rapidly with small breaks in the cutaneous barrier and red streak or swelling associated with fever. Can lead to fibrosis and lymphedema. Tinea pedis Dry scaly pattern on the periphery of the foot. Can result in moist erosions and bullae on the soles of the feet and between the toes. Oxalosis Autosomal recessive hereditary disease due to faulty metabolism of glyoxylic acid. Calcium oxalate is deposited in body tissues. Cryoglobulinemia Symptoms may include mild recurrent lower extremity purpura, joint pains and swelling, enlargement of the spleen, vasculitis skin with purplish patches, weight loss, glomerulonephritis, and peripheral neuropathy. Livediod vasculitis Presents as purpuric macules and papules that progress to small, tender irregular ulcers of lower extremities. Ulcers heal with stellate ivory white atrophic plaques. Pyoderma gangrenosum Ragged bluish-red purulent ulcer with overhanging edges and a necrotic base around the edge of the lesions, usually occurring on the lower extremities. Lipodermatosclerosis Painful, tender condition of the leg just above the ankle. The skin becomes brown, smooth, and tight. Can lead to intensely fibrotic ulcers. Metastatic calcinosis Spreading abnormal deposition of lime salts in the tissues. Firm, tender papules, nodules, or palques that may discharge chalky material. Necrotizing fasciitis Trauma at site, discomfort, increased pain out of proportion to injury; flu-like symptoms; swollen, hot tissue with redness; sunburn-type rash; large and then dark, boil-like blister becoming gangrenous and necrotic. Pseudoxanthoma elasticum Condition resembling xanthoma that is a chronic degenerative cutaneous disease marked by yellow patches and stretching of skin.
Acknowledgment: The author would like to give thanks to Pat Weiskittel, MSN, RN, CNN, Renal Transplant Coordinator, for review of the manuscript at the University of Cincinnati.
Angelis, M., Wong, L.L., Myers, S.A., & Wong, L.M. (1997). Calciphylaxis in patients on hemodialysis: A prevalence study. Surgery, 122(6), 1083-1089.
Asirvatham, S., Sebastian, C., Sivaram, C.A., Kaufman, C., & Chandrasekaran, K. (1998). Aortic valve involvement in calciphylaxis: Uremic small artery disease with medial calcification and intimal hyperplasia. American Journal of Kidney Diseases, 32(3) 499-502.
Bleyer, A.J., Choi, M., Igwemezie, B., de la Torre, E., & White, W.L. (1998). A case control study of proximal calciphylaxis. American Journal of Kidney Diseases, 32(3), 376-383.
Brown, D.F., Denney, C.F., & Burns, D.K. (1998). Systemic calciphylaxis associated with massive gastrointestinal hemorrhage. Archives of Pathology and Laboratory Medicine, 122(7), 656-659.
Edwards, R.B., Jaffe, W., Arrowsmith, J., & Henderson, H.P. (2000). Calciphylaxis: A rare limb and life-threatening cause of ischaemic skin necrosis and ulceration. British Journal of Plastic Surgery, 53(3), 253-255.
Essary, L.R., & Wick, M.R. (2000). Cutaneous calciphylaxis: An under recognized clinicopathologic entity. American Journal of Clinical Pathology, 113(2), 280-287.
Flanigan, K.M., Bromberg, M.B., Gregory, M., Baringer, F.R., Jones, C.R., Nester, T.A., Klatt, E.C., & Townsend, J.J. (1998). Calciphylaxis mimicking dermatomyositis: Ischemic myopathy complicating renal failure. Neurology, 51(6), 1634-1640.
Goldsmith, D.J.A. (1997). Diabetes, tissue infarction, and calciphylaxis. American Journal of Medicine, 102(2), 171-172.
Green, J.A., Green, C.R., & Minott, S.D. (2000). Calciphylaxis treated with neurolytic lumbar sympathetic block: Case report and review of the literature. Regional Anesthesia and Pain Medicine, 25(3), 310-312.
Handa, S.P., & Strzelczak, D. (1998). Uremic small artery disease: Calciphylaxis with penis involvement. Clinical Nephrology, 50(40), 258-261.
Ivker, R.A., Woosley, J., & Briggaman, R.A. (1995). Calciphylaxis in three patients with end-stage renal disease. Archives of Dermatology, 131(1), 63-68.
James, L.R., Lajoie, G., Prajapati, D., Gan, B.S., & Bargman, J.M. (1999). Calciphylaxis precipitated by ultraviolet light in a patient with end-stage renal disease secondary to systemic lupus erythematous. American Journal of Kidney Disease, 34(5), 932-936.
Khafif, R.A., DeLima, C., Silverberg, A. & Frankel, R. (1990). Calciphylaxis and systemic calcinosis: Collective review. Archives of Internal Medicine, 150(5), 956-959.
Lipsker, D., Chosidow, O., Martinez, F., Challier, E., & Frances, C. (1997). Low-calcium dialysis in calciphylaxis. Archives of Dermatology, 133(6), 798-780.
McAuley, K., Devereux, E, & Walker, R. (1997). Calciphylaxis in two noncompliant patients with end-stage renal failure. Nephrology, Dialysis, Transplantation, 12(5), 1061-1063.
Oh, D.H., Eulau, D., Tokugawa, D.A., McGuire, J.S., & Kohler, S. (1999). Five cases of calciphylaxis and a review of the literature. Journal of the American Academy of Dermatology, 40(6), 979-987.
Randall, D.P., Fisher, M.A., & Thomas, C. (2000). Rhabdomyolysis as the presenting manifestation of calciphylaxis. Muscle & Nerve, 23(2), 289-293.
Sankarasubbaiyan, S., Scott, G., & Holley, J.L. (1998). Cryofibrinogenemia: An addition to the differential diagnosis of calciphylaxis in end-stage renal disease. American Journal of Kidney Diseases, 32(3), 494-498.
Selye, H. (1962). Calciphylaxis. Chicago: University of Chicago Press.
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Calcyphylaxis: What Nurses Need to Know
By Deborah E. Bliss, MSN, RN
Posttest--2.0 Contact Hours Posttest Questions
(See posttest instructions on the answer form, next page) The Editor gratefully acknowledges Charlotte Szromba, MSN, RN, NP, CS, CNN for writing portions of this posttest.
1. Calciphylaxis, more recently known as vascular calcification-cutaneous necrosis syndrome, has also been described as:
A. uremic gangrene syndrome only.
B. uremic gangrene syndrome and metastatic calcinosis only.
C. uremic gangrene syndrome, metastatic calcinosis, and azotemic calcific arteriopathy only.
D. uremic gangrene syndrome, metastatic calcinosis, azotemic calcific arteriopathy, and intimal hyperplasia.
2. Mortality rates for individuals with proximal calciphylaxis are:
A. 10 to 30%.
B. 31 to 50%.
C. 60 to 80%.
D. 81 to 100%.
3. In his study of rats, Selye concluded that calciphylaxis is a combination of factors, including induced hypersensitivity in which tissues react to challenging agents. In applying this theory to humans, some medications that could be "challengers" are:
A. immunosuppressive agents and warfarin.
B. calcium-based phosphate binders and vitamin E.
C. oral iron compounds, and chelating agents for aluminum removal.
D. folic acid and prednisone.
4. Which of the following statement is correct regarding the "sensitizing" factors involved in the development of calciphylaxis in humans?
A. Local trauma and hypocoagulable states are contributing factors.
B. High calcium-phosphate product and hyperphosphatemia are components of sensitization.
C. Medications serve as components of sensitization.
D. ESRD and hypoparathyroidism are contributing factors.
5. The etiology of calciphylaxis is unclear, however it is thought to be multi-factorial. The most prominent characteristic is:
A. alteration in calcium/phosphorous metabolism.
B. long periods on dialysis.
D. preexisting hypercoaguable state.
6. Lester W. is an African American weighing 112.0 kg who is 51 years old, and on hemodialysis for the past 3 years with BP of 118/78. Diagnostic labs include: calcium/phosphorous product of 53, PTH 109, and albumin 3.0 mg/dl. What factors put this individual at risk for calciphylaxis?
A. Race and hypotension.
B. Calcium/phosphorous product and hyperparthyroidism.
C. Obesity and low albumin level.
D. Age and length of time on hemodialysis.
7. The earliest presenting sign for calciphylaxis is:
A. plagues in the dermis.
B. nodules in the subcutaneous tissue.
C. ulcerated digits.
D. livedo reticularis.
8. Confirmed diagnosis of calciphylexis can be made with:
A. X-ray showing calcification of arteries.
B. inflammation of the vessel verified on skin biopsy.
C. skin biopsy showing fibrous thrombi occluding vessel lumina.
D. lab test showing hyperparathyroidism.
9. Your patient complains of nonhealing ulcers. Calciphylaxis is the probable diagnosis. What other finding would you expect to be present?
B. Absent pulses.
C. Swelling of extremity with fever.
D. Proximal ulcers.
10. Abby C. has been on hemodialysis for 10 years, with poor adherence to phosphate binding therapy. She presents to the dialysis clinic with a nonhealing ulcer on her left leg. Her ESRD was caused by diabetes. What should be included in the differential diagnoses?
B. Calciphylaxis and peripheral vascular disease.
C. Calciphylaxis, peripheral vascular disease, and cellulitis.
D. Calciphylaxis, peripheral vascular disease, cellulitis, and tinea pedis.
11. You review Abby's history and note the following: Height 5 feet, 6 inches; weight 150 pounds; and body mass index 24. Lab reveals: PTH level 1700; calcium 10.2 mg/dl; phosphorus 8.8 mg/dl; albumin 4.0 mg/dl; and Hgb A1C 7.0. Based on the information, what instructions would you give to Abby C. concerning prevention of calciphylaxis?
A. "Take calcium carbonate to control your phosphorous."
B. "It is important that you lose weight."
C. "Increase your protein intake."
D. "Limit high phosphorous foods in your diet."
12. The treatment of choice for Abby C. is:
B. local wound care.
C. hyperbaric oxygen therapy.
D. aggressive control of calcium phosphorous product.
Calcyphylaxis: What Nurses Need to Know
By Deborah E. Bliss, MSN, RN
* Select the best answer and circle the appropriate letter on the answer grid below.
* Complete the evaluation.
* Send only the answer form to the ANNA National Office; East Holly Avenue Box 56, Pitman, NJ 08071-0056.
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ANNA Member--$15 Non-Member--$25 Expiration Date -- (from membership card)
Complete the Following
ANNA Member -- Yes -- No CNN -- Yes -- No
Source of Article:  Journal  ANNA Web site (ANNAlink)
Note: If you wish to keep the journal intact, you may photocopy the answer sheet or access this posttest at www.annanurse.org
Posttest Answer Grid
Please circle your answer choice:
1. a b c d
2. a b c d
3. a b c d
4. a b c d
5. a b c d
6. a b c d
7. a b c d
8. a b c d
9. a b c d
10. a b c d
11. a b c d
12. a b c d
Goal: Recognize the risk factors for and early signs of calciphylaxis in order to initiate prompt treatment.
Strongly Strongly Evaluation disagree agree 1. The objectives were related to the goal. 1 2 3 4 5 2. Objectives were met a. Describe the pathogenesis and 1 2 3 4 5 etiology of calciphylaxis. b. Identify risk factors and clinical 1 2 3 4 5 manifestations, including differential diagnosis. c. Discuss prevention and treatment of 1 2 3 4 5 calciphylaxis and the role of the nephrology nurse. 2. The teaching/learning resources were 1 2 3 4 5 effective to complete this activity. 3. A self-study format was effective for 1 2 3 4 5 the content. 4. The On-Line format was effective. (if 1 2 3 4 5 downloaded) 5. Minutes required to complete self-study, including the posttest: -- minutes
What topics do you need: --
Deborah E. Bliss, MSN, RN, is Cardiovascular Nurse Practitioner/Research Study Coordinator, Cincinnati Veterans Affairs Medical Center, Cincinnati, OH. She has a master's degree in adult health, acute care nursing from the University of Cincinnati and is certified as an acute care nurse practitioner.
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|Author:||Bliss, Deborah E.|
|Publication:||Nephrology Nursing Journal|
|Date:||Oct 1, 2002|
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