CALCIPHYLAXIS
CALCIPHYLAXIS ICD-10: E83.59
• Calciphylaxis is characterized by progressive cutaneous tissue ischemia and necrosis associated with small-vessel vasculopathy with endovascular calcification, intimal proliferation and fibrin thrombi.
• It occurs most often in the setting of end-stage renal disease,Ýiabetes mellitus, secondary hyperparathyroidism and warfarin use.
• Precipitating factors: Warfarin, corticosteroids, 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. Parathyroid hormone 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, sodium thiosulfate infusions/intralesional (infusions can be complicated by metabolic acidosis), partial parathyroidectomy when indicated, hyperbaric oxygen therapy, bisphosphonates, and debridement of necrotic tissue.
A B
A B
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 (A) A large ulcerated and painful lesion is noted in the medial thigh; the surrounding skin is indurated and hairless. The proximal purpuric area is an “earlier” lesion that became ulcerative six weeks later. (B) Surrounding purpura is noted with early bulla formation and ulceration. The patient complained mostly of pain. (Used with permission of Dr. Kenneth Greer.)