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CLASSIFICATION OF SKIN CHANGES

  • Acute renal failure

    • Edema

    • Uremic frost (deposition of urea crystals on the skin's surface in severe uremia)

  • Chronic renal failure

    • Edema

    • Uremic frost

    • Calciphylaxis

    • Bullous disease of hemodialysis (pseudoporphyria, see Section 23)

    • Nephrogenic fibrosing dermopathy

    • Acquired perforating dermatosis

CALCIPHYLAXIS ICD-10: E83.59

  • Calciphylaxis is characterized by progressive cutaneous necrosis associated with small- and medium-sized vessel vasculitis, calcification, and thrombosis.

  • It occurs in the setting of end-stage renal disease, diabetes mellitus, and secondary hyperparathyroidism. Most often follows initiation of hemo or peritoneal dialysis.

  • Precipitating factors: glucocorticosteroids, albumin infusions, IM tobramycin, iron dextran complex, calcium heparinate, and vitamin D.

  • Preinfarctive lesions show mottling or a livedo reticularis pattern, dusky red (Fig. 18-1A).

  • Turn into black, leathery eschar (Fig. 18-1B) and ulcer with tightly adherent black or leathery slough. Ulcers enlarge over weeks to months; when debrided reach down to fascia and beyond; areas of plate-like induration can be palpated surrounding infarcted or ulcerated lesions (Fig. 18-2).

  • Extremely painful.

  • Lower extremities, abdomen, buttocks, and penis.

  • Azotemia. Calcium X phosphate ion product usually elevated. Parathormone levels usually but not always elevated. Dermatopathology: Calcification of media of small- and medium-sized blood vessels in dermis and subcutaneous tissues.

  • Slowly progressive, despite therapy. Ulcers become secondarily infected.

  • Management: Treatment of renal failure, partial parathyroidectomy when indicated, and debridement of necrotic tissue.

Figure 18-1

Calciphylaxis (A) 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 indurated and represents a plate-like subcutaneous mass that is appreciated only upon palpation.

Figure 18-2

Calciphylaxis, extensive Lesions are ulcerated; the surrounding skin is indurated and best seen on left thigh where skin is hairless. Similar lesions are also found on the abdomen.

NEPHROGENIC FIBROSING DERMOPATHY (NFD) ICD-10: L90.8

  • NFD is a fibrosing disorder in patients with acute or chronic renal failure.

  • Most patients receive hemodialysis or peritoneal dialysis; in acute renal failure, NFD occurs without dialysis.

  • It is part of a wider spectrum of nephrogenic systemic fibrosis involving the heart, lungs, diaphragm, skeletal muscle, liver, genitourinary tract, and central nervous system.

  • Etiology unknown but exposure to gadodiamide containing contrast media for MR angiography is a strong association. Gadodiamide is found only in lesions and not in normal tissue.

  • NFD is characterized by acute onset, brawny indurations, plaque-like or nodular, bound down upon palpation (Fig. 18-3); up to 20 cm and more in diameter, ...

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