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Calciphylaxis Workup

  • Author: Julia R Nunley, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Feb 08, 2016
 

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.[33]

Radiologic findings of a hand in a patient with caRadiologic 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.[34] Serial bone scanning can also possibly be used to monitor progression or regression of disease.[35, 36]

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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.[37] 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 (see images below). A mixed inflammatory infiltrate frequently occurs. Subcutaneous calcium deposits with panniculitis and fat necrosis may sometimes be found. Vascular microthrombi are frequently evident.

This image shows circumferential medial calcific dThis image shows circumferential medial calcific deposits obliterating the external elastica of an arteriole. Histologic images courtesy of Steve A. McClain, MD, Department of Dermatology SUNY-Stony Brook.
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Contributor Information and Disclosures
Author

Julia R Nunley, MD Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, Women's Dermatologic Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Board of Dermatology<br/>Co-Editor for the text Dermatological Manifestations of Kidney Disease .

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Smeena Khan, MD Private Practice, Adult and Pediatric Dermatology Associates

Smeena Khan, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

References
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Several lesions of calciphylaxis that occurred on the lower extremity of a patient undergoing dialysis. These lesions developed in areas of livedo reticularis and followed the path of the vasculature.
An isolated lesion of calciphylaxis manifesting as an enlarging necrotic plaque on the lower extremity of a patient undergoing dialysis. The stellate purpuric morphology can be appreciated surrounding the area of necrosis.
Calciphylaxis may manifest as rapidly progressive, diffuse and extensive, cutaneous necrosis, as is seen in this patient with chronic renal failure. Bullae may also be seen as a rare manifestation of calciphylaxis.
Radiologic findings of a hand in a patient with calciphylaxis. Extensive calcification of the radial and ulnar arteries is readily visible.
Histologically, calcification of the blood vessels, as well as the subcutis, can be seen in calciphylaxis.
Demonstrated here is the characteristic circumferential medial calcific deposit in an arteriole with subintimal edema. Histologic images courtesy of Steve A. McClain, MD, Department of Dermatology SUNY-Stony Brook.
This image shows circumferential medial calcific deposits obliterating the external elastica of an arteriole. Histologic images courtesy of Steve A. McClain, MD, Department of Dermatology SUNY-Stony Brook.
 
 
 
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