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  icon Rare
  icon Not so common
  icon Common
  icon Low morbidity
  icon Considerable morbidity
  icon Serious

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  • Acute renal failure
    • Edema
    • Uremic frost (deposition of urea crystals on skin surface in severe uremia)
  • Chronic renal failure
    • Edema
    • Uremic frost
    • Calciphylaxis
    • Bullous disease of hemodialysis (pseudoporphyria, see Section 22)
    • Nephrogenic fibrosing dermopathy
    • Acquired perforating dermatosis

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ICD-9 : 275.49   Image not available. Image not available. Image not available. Image not available.

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  • Calciphylaxis is characterized by progressive cutaneous necrosis associated with small- and medium-sized vessel calcification.
  • It occurs in the setting of end-stage renal disease, diabetes mellitus, and hyperparathyroidism.
  • Preinfarctive lesions show mottling or livedo reticularis pattern, dusky red.
  • Turn into black, leathery eschar.
  • Extremely painful.
  • Extend to fascia and beyond.
  • Lower extremities, abdomen, buttocks, penis.

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Epidemiology

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Age of Onset

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Middle to old age.

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Sex

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Equal.

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Pathogenesis

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The pathogenesis is poorly understood. While vascular calcifications are common in patients with chronic renal failure and are asymptomatic, in calciphylaxis, there is sudden thrombosis in calcified vessels. In animal models, calciphylaxis is described as a condition of induced systemic hypersensitivity in which tissues respond to appropriate challenging agents with calcium deposition. Calciphylaxis is associated with chronic renal failure, secondary hyperparathyroidism, and an elevated calcium phosphate end product. Implicated “challenging agents” include glucocorticoids, albumin infusions, intramuscular tobramycin, iron dextran complex, calcium heparinate, immunosuppressive agents, and vitamin D.

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Clinical Manifestation

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Even early infarctive lesions are exquisitely tender and painful.

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Disease Associations

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Occurs in end-stage renal disease. Onset often closely follows initiation of hemo- or peritoneal dialysis. Most patients are diabetic. Hyperparathyroidism.

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Skin Lesions

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Initially preinfarctive ischemic plaques occur, appearing as mottling or having a livedo reticularis pattern, dusky red to violaceous (Fig. 17-1A). Bullae may form over ischemic tissue, which eventually becomes necrotic. Central infarcted sites have a tightly adherent black or yellowish, leathery slough (Fig. 17-1B). Lesions gradually enlarge over weeks to months; when debrided, deep ulcers reaching down to the fascia result (Fig. 17-2). Ischemic skin frequently becomes secondarily infected; infection can remain localized or become invasive, causing cellulitis and bacteremia. In addition, large areas of induration can be defined on palpation as platelike subcutaneous masses that extend beyond infarcted or ulcerated areas (Fig. 17-2).

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Figure 17-1
Graphic Jump LocationGraphic Jump Location

CalciphylaxisA.Early stage. An area of mottled erythema, starburst-like and reminiscent of livedo reticularis with two small ulcerations. Patient has chronic renal failure and is on hemodialysis. Even at this early stage lesions are extremely painful. B. Calciphylaxis, more advanced lesion. An area of jagged necrosis on the lower leg in a patient with diabetes and chronic renal failure who is on hemodialysis. The surrounding skin is ...

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