Ailments of Unknown Cause Codexery

Calciphylaxis

Rare syndrome of painful skin lesions from small vessel calcification.

Last updated

Calciphylaxis is a rare syndrome causing painful skin lesions, also known as calcific uremic arteriolopathy.[1] It involves calcification of small blood vessels in fat and deep skin layers, leading to blood clots and skin cell death.[1] The disease is seen mostly in end-stage kidney disease patients and has a poor prognosis, with typical life expectancy under one year.[1]

Key facts and history

It is not a hypersensitivity reaction.[2] The current belief is that abnormal calcium and phosphate homeostasis in end-stage kidney disease causes calcium deposition in vessels.[2] A thrombotic event then occludes the vessel, causing tissue infarction.[2] Reported risk factors include female sex, obesity, and medications such as warfarin.[3]

No one has identified a specific cause for calciphylaxis.[2] The earliest visible signs on the skin are a blotchy, net-like pattern (livedo reticularis) accompanied by hardening of the tissue.[4] These sores are invariably very painful and are typically located on the lower limbs, belly, rear end, and male genitalia.[4] A large number of instances result in a deadly, whole-body bacterial infection.[5]

For individuals with end-stage kidney disease, the chance of dying within one year after diagnosis ranges from 45% to 80%.[6]

What the cited sources add

The Wikipedia entry for calciphylaxis uses the source "Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology" (McGraw Hill, 2013) to support the claim that numerous cases progress to systemic bacterial infection and death.[5][7]

References

  1. Wikipedia, "Calciphylaxis" (CC BY-SA 4.0), which cites: Nigwekar, "Calciphylaxis", New England Journal of Medicine, May 2018, p. 1704–1714; Nigwekar, "Calciphylaxis", New England Journal of Medicine, May 2018, p. 1704–1714; Angelis, "Calciphylaxis in patients on hemodialysis: A prevalence study", Surgery, 1997, p. 1083–1090; Nigwekar, "Calciphylaxis", New England Journal of Medicine, May 2018, p. 1704–1714 (original not read by us) en.wikipedia.org
  2. Wikipedia, "Calciphylaxis" (CC BY-SA 4.0), which cites: Wilmer, "Calciphylaxis: Emerging Concepts in Prevention, Diagnosis, and Treatment", Seminars in Dialysis, 2002, p. 172–186 (original not read by us) en.wikipedia.org
  3. Wikipedia, "Calciphylaxis" (CC BY-SA 4.0), which cites: Arseculeratne, "Calciphylaxis – a topical overview", Journal of the European Academy of Dermatology and Venereology, 2006, p. 493–502; Lal, "Determinants of survival in patients with calciphylaxis: A multivariate analysis", Surgery, December 2009, p. 1028–1034 (original not read by us) en.wikipedia.org
  4. Wikipedia, "Calciphylaxis" (CC BY-SA 4.0), which cites: "Calciphylaxis", The Lancet, 2014, p. 1067 (original not read by us) en.wikipedia.org
  5. Wikipedia, "Calciphylaxis" (CC BY-SA 4.0), which cites: Wolff, "Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology", McGraw Hill, 2013-03-06, p. 429 (we also read that source) en.wikipedia.org
  6. Wikipedia, "Calciphylaxis" (CC BY-SA 4.0), which cites: Nigwekar, "Calciphylaxis", New England Journal of Medicine, May 2018, p. 1704–1714; Bajaj, "Calciphylaxis in Patients With Normal Renal Function: A Case Series and Systematic Review", Mayo Clinic Proceedings, September 2018, p. 1202–1212 (original not read by us) en.wikipedia.org
  7. Open Library record, ISBN 9780071793025 ("Fitzpatricks Color Atlas and Synopsis of Clinical Dermatology Seventh Edition 7th Edition"). CC0 (metadata); book content not read. openlibrary.org

Each numbered sentence above was checked against the source it cites before publication. Sentences citing Wikipedia adapt text from the English Wikipedia article Calciphylaxis (contributors), used under CC BY-SA 4.0; this entry is available under the same licence. Other sources were used for facts only; no text or images are copied from them.

More in Ailments of unknown cause

Cholinergic urticariaCluster headacheCopenhagen diseaseDancing maniaAcrocyanosisAcute interstitial pneumonitisAttention deficit hyperactivity disorderBaby colicBell's palsyBenign paroxysmal vertigo of childhoodBlain (animal disease)Thromboangiitis obliterans

Sources

Compiled from Wikipedia and the sources listed below. Text from Wikipedia is available under CC BY-SA 4.0; this entry is adapted from it.

Spotted an error? Know more?

Reader corrections go straight into our review queue. Suggest an edit · How this site is sourced

Comments

Loading…
Open in the interactive codex →