Skin nontumor
Panniculitis
Calciphylaxis
Last author update: 1 July 2011
Last staff update: 28 August 2020
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PubMed Search: Calciphylaxis [title]
Table of Contents
Definition / general | Etiology | Clinical features | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosis | Additional referencesCite this page: Hamodat M. Calciphylaxis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorcalciphylaxis.html. Accessed April 19th, 2024.
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
- 69 year old woman with primary hyperparathyroidism due to parathyroid adenoma (Arch Pathol Lab Med 2001;125:1351)
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
Microscopic (histologic) 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
Microscopic (histologic) images
Contributed by Sepideh N. Asadbeigi, M.D.
Calcium deposits
Calcium deposits in deep dermis
Calciphylaxis
involving small
blood vessels with
associated thrombi
Subcutaneous fat with vascular calcium deposits
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
