ReportCalciphylaxis: Natural history, risk factor analysis, and outcome
Section snippets
Methods
The study was approved by the Institutional Review Board of Mayo Foundation and consisted of a retrospective review of the medical records of 64 patients in whom calciphylaxis was diagnosed at Mayo Clinic, Rochester, Minnesota, over an 11-year period (1992 to 2002). This included 16 patients in the previous study of Kang et al.1 Of the 64 patients, 49 (77%) were receiving dialysis for end-stage renal failure (“dialysis patients”). The remaining 15 patients were designated “nondialysis
Results
Follow-up information was available for 63 of the 64 patients with calciphylaxis, of whom 51 (81%) had died by the completion of the study. The median survival from the date of diagnosis for these 63 patients was 2.64 months (range, 0-7 years). Overall and group-specific survival curves are presented in Fig 1. At the time of death, calciphylaxis-related cutaneous ulceration was present in 34 patients (67%), and the estimated cause-specific survival rate at 1 year was 45.8%. Sepsis was a
Discussion
In 1961, Selye, Gentile, and Prioreschi2 defined “calciphylaxis” as a systemic hypersensitivity reaction analogous to an allergic reaction (anaphylaxis). In experiments on rodents, Selye and coworkers induced calcification of various organs (including the skin) after animals had been “sensitized” with one of several agents they referred to as “calcifiers” (eg, dihydrotachysterol, vitamin D2, vitamin D3, and parathyroid hormone), followed by exposure to a “challenger” (eg, metallic salts such as
References (24)
- et al.
Calcifying panniculitis with fat and skin necrosis in a case of uraemia with autonomous hyperparathyroidism
Lancet
(1968) - et al.
The vascular calcification-cutaneous necrosis syndrome
J Am Acad Dermatol
(1995) - et al.
Calcified subcutaneous arterioles with infarcts of the subcutis and skin (“calciphylaxis”) in chronic renal failure
Am J Kidney Dis
(2000) - et al.
Cutaneous necrosis from calcific uremic arteriolopathy
Am J Kidney Dis
(1998) - et al.
Gene expression of glucocorticoid receptor α and β in giant cell tumour of bone: evidence of glucocorticoid-stimulated osteoclastogenesis by stromal-like tumour cells
Mol Cell Endocrinol
(2001) - et al.
Skin necrosis associated with acquired protein C deficiency in patients with renal failure and calciphylaxis
Am J Med
(1990) - et al.
Calciphylaxis and antiphospholipid antibody syndrome
J Am Acad Dermatol
(2000) - et al.
Is calciphylaxis best treated surgically or medically?
Surgery
(2000) - et al.
Cutaneous molt induced by calciphylaxis in the rat
Science
(1961) - et al.
Calcific small vessel ischemic disease (calciphylaxis) in dialysis patients
Int J Artif Organs
(2000)
[Nanobacteria as a cause of renal diseases and vascular calcifying pathology in renal patients (“endovascular lithiasis”).]
Enferm Infecc Microbiol Clin
Uraemic gangrene syndrome
Acta Derm Venereol
Cited by (419)
Penile calciphylaxis with penoscrotal necrosis: A case report with literature review
2023, Urology Case ReportsA case of nonuremic calciphylaxis of the penis responding to sodium thiosulfate therapy
2023, JAAD Case ReportsCalciphylaxis and Kidney Disease: A Review
2023, American Journal of Kidney DiseasesUremic calciphylaxis
2022, Nephrologie et TherapeutiqueOcclusive cutaneous vasculopathies as cause of chronic ulcers
2024, JDDG - Journal of the German Society of Dermatology
Funding sources: None.
Conflicts of interest: None identified.
- ∗
Visiting Medical Student, Mayo Clinic College of Medicine.