1. Renal insufficiency
3. Female sex
5. Hypotensive episode
6. Recent period of rapid weight loss
9. Albumin infusions
10. Increased parathyroid hormone level
11. Vitamin D use
12. Calcium carbonate use
13. Increased calcium-phosphate product
14. Warfarin use
15. Protein C or S deficiency or functional impairment
Patients with calciphylaxis usually present with painful indurated plaques or nodules of the abdomen, thigh, and hips, which frequently ulcerate (Figures 35.3 and 35.4). Livedo reticularis, purpura, and bullae may also be seen. Disease localized to the breast or penis has been described (11,17). There is also a distal form of the disease in which patients present with acral ischemia resembling that of peripheral arteriosclerotic disease. A distinguishing feature is that peripheral pulses are generally intact. These patients are reported to have higher survival rates than those with proximal disease (18).
In addition to cutaneous involvement in calciphylaxis, myopathy may develop secondary to skeletal muscle involvement. This usually occurs in areas of skin involvement, and may result in rhabdomyolysis. Other less common areas of involvement are heart, joints, lungs, pancreas, eyes, and gastrointestinal tract (11).
Histopathologic features of calciphylaxis are vascular calcification and varying degrees of necrosis and inflammation, depending on timing and location of the biopsy specimen. Calcification occurs in small to medium-sized venules, and arterioles, in the subcutaneous tissue, and in some instances, the dermis (Figure 35.5A and 35.5B) (18,19). Calcification of subcutaneous septate and adipocytes may also occur (14). The inflammatory infiltrate consists of lymphocytes, neutrophils, and occasional eosinophils. Early lesions devoid of necrosis may have a dermal inflammatory infiltrate. The subcutaneous component is predominantly septal. Calcifications are usually readily seen, but occasionally require histochemical staining with von Kossa or Alizarin red. The degree of calcium deposition identified microscopically does not appear to
Figure 35.3. Reticulated and stellate pattern of ulceration with eschar formation on the legs.
correlate with clinical severity. Vascular thrombi are seen in the minority of cases (19). Necrosis is variable. Subcutaneous calciphylaxis-like calcifications have been reported in biopsy specimens from patients with nephrogenic fibrosing dermopathy, a morphea-like cutaneous scleros-ing disorder occurring in some patients with renal failure. This finding was not accompanied by clinical evidence of calciphylaxis (20). These calcifications may correspond to the "primary lesion" of calciphylaxis that represents the setting in which clinical lesions develop. The histologic differential diagnosis of subcutaneous calcifications includes pancreatic panniculitis and Monckeberg's medial calcific sclerosis. Pancreatic panniculitis has diffuse subcutaneous necrosis with "ghost" cells and an infiltrate of neutrophils, without vascular calcification. Monckeberg's medial calcific sclerosis occurs as an incidental finding in cutaneous biopsy specimens from the leg, and correlates with underlying arteriosclerotic disease.
The differential diagnosis of calciphylaxis depends upon the distribution of lesions, but may include warfarin
necrosis, primary hyperoxaluria, peripheral arteriosclerotic disease, vasculitis, emboli, cryoglobulinemia, and cryofibrinogenemia. The diagnosis of calciphylaxis in the right clinical setting is usually apparent. However, some evaluation is indicated for confirmation, and perhaps more importantly, to gather data that will help direct management. A diagnostic evaluation is suggested in the following:
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