Calciphylaxis Workup

Updated: Aug 02, 2018
  • Author: Julia R Nunley, MD; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

Consider the following laboratory tests and results:

  • Serum blood urea nitrogen and creatinine levels

  • Serum calcium, phosphate, alkaline phosphatase, and albumin levels

  • Serum parathyroid hormone (PTH) level

  • Coagulation factors - Prothrombin time, activated partial thromboplastin time, antithrombin III, protein C level, protein S level, anticardiolipin level, lupus anticoagulant level, factor V Leiden level, and homocysteine level

  • Cryoglobulin and rheumatoid factor measurements

  • Hepatitis C antibody level

  • Cryofibrinogen level

  • Serum amylase and lipase level

  • Aluminum level

  • Measures of inflammation - Erythrocyte sedimentation rate and C-reactive protein value

  • To exclude underlying vasculitis - Antineutrophilic antibody (ANA), antineutrophil cytoplasmic antibodies (ANCA)

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Imaging Studies

Plain radiography uniformly demonstrates an arborization of vascular calcification within the dermis and the subcutaneous tissue (see the image below). Although calcification is common in persons with end-stage renal disease, and not specific for calciphylaxis, a recent study showed patients with calciphylaxis had more vascular calcifications, more small vessel calcifications, and a netlike pattern of calcifications. This netlike pattern, when present, was strongly associated with the presence of calciphylaxis. [36]

Radiologic findings of a hand in a patient with ca Radiologic findings of a hand in a patient with calciphylaxis. Extensive calcification of the radial and ulnar arteries is readily visible.

Bone scintigraphy may be used as a noninvasive diagnostic tool because the bone matrix protein osteopontin has recently been demonstrated in calciphylaxis lesions. [37] Serial bone scanning can also possibly be used to monitor progression or regression of disease. [38, 39]

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Procedures

Punch biopsies may not be adequate because the quantity or depth of tissue obtained may not be enough for diagnosis. An incisional cutaneous biopsy is usually diagnostic. [40] Ample subcutaneous tissue must be available for adequate evaluation.

The decision to perform a biopsy on a nonulcerated lesion should not be made lightly because it could result in a nonhealing wound. Although lesions of calciphylaxis have a clinical appearance suggestive of avascular necrosis, the tissue often bleeds freely during surgery. Furthermore, lesional ulceration increases the mortality rate 2-fold.

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Histologic Findings

Biopsy specimens typically demonstrate calcification within the media of small- and medium-sized arterioles with extensive intimal hyperplasia and fibrosis, but this also may be seen in uninvolved skin in patients with chronic kidney disease or atherosclerosis. [41] A mixed inflammatory infiltrate frequently occurs. Subcutaneous calcium deposits with panniculitis and fat necrosis may sometimes be found. Vascular microthrombi are frequently evident. See the image below.

Calcification of capillaries within the subcutaneo Calcification of capillaries within the subcutaneous fat, characteristic of calciphylaxis. Image courtesy of Dirk Elston, MD.
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