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Skin-nontumor / Clinical Dermatology

Other dermatoses

Calciphylaxis

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 19 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● 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
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● 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
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● 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%

Treatment
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● Surgical removal of autonomous parathyroid gland (if due to primary hyperparathyroidism)
● Also hyperbaric oxygen, antihistamines, corticosteroids

Case reports
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● 69 year old woman with primary hyperparathyroidism due to parathyroid adenoma (Arch Pathol Lab Med 2001;125:1351)

Clinical images
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Necrosis resembling a burn injury

Calciphylaxis on abdomen of patient with end stage renal disease

Micro description
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● 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
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Various images

Differential diagnosis
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● 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
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● eMedicine

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Calciphylaxis

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