Skin-nontumor / Clinical dermatology
Other dermatoses
Calciphylaxis

Author: Mowafak Hamodat, M.D., MB.CH.B, MSc., FRCPC (see Authors page)

Revised: 23 June 2016, last major update July 2011

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Calciphylaxis [title]

Cite this page: Calciphylaxis. PathologyOutlines.com website. http://pathologyoutlines.com/topic/skinnontumorcalciphylaxis.html. Accessed December 7th, 2016.
Definition / General
  • Rare, life-threatening condition of progressive cutaneous necrosis due to small and medium sized vessel calcification
  • Also called calcifying panniculitis or vascular calcification-cutaneous necrosis syndrome
Etiology
  • Due to primary, secondary or tertiary hyperparathyroidism and associated elevated calcium and phosphate levels, which exceed their solubility and deposit in vessels, causing progressive vascular compromise in skin and subcutaneous fat, and less often muscle
Clinical Features
  • Lesions are bilateral and symmetric, pruritic and frequently painful / tender
  • Often lower extremity, with preservation of pulses
  • Less often affects the breast, abdomen and penis
  • Mortality rate up to 60%
Case Reports
Treatment
  • Surgical removal of autonomous parathyroid gland (if due to primary hyperparathyroidism)
  • Also hyperbaric oxygen, antihistamines, corticosteroids
Clinical Images

Images hosted on other servers:

Necrosis resembling a burn injury

Calciphylaxis on abdomen of patient with end stage renal disease

Micro Description
  • Involves intima and media of large and small blood vessels
  • Deposits are associated with thrombi
  • Intimal fibroblastic proliferation with luminal narrowing has also been described
  • Also hemorrhage within the subcutaneous fat, fat necrosis accompanied by lobular lymphohistiocytic infiltrate
  • Interstitial calcification is rare
  • Pseudoxanthoma elasticum like changes may occur
Micro Images

Images hosted on other servers:

Various images

Differential Diagnosis
  • Metastatic calcification: due to hyperparathyroidism or chronic renal failure
  • Dystrophic calcinosis cutis: deposits in areas of previously abnormal skin
  • Tumoral calcinosis: no known cause
  • Idiopathic calcinosis of scrotum
  • Subepidermal calcified nodule: childhood papule, small, fleshy, firm, on face
Additional References