Revision 2 for 'Calciphylaxis'

Calciphylaxis, or calcific uremic arteriolopathy, is a rare condition, involving subcutaneous vascular calcification and cutaneous necrosis (small blood vessels of the fat tissue and the skin), which is seen most often in patients with renal failure (this is not an absolute requirement).

Some authors describe as a syndrome of vascular calcification, thrombosis and skin necrosis.

Epidemiology

Clinical presentation

Pathophysiology

Treatment and prognosis

Etymology

First described in 1898 by Bryant and White.

  In 1962 that the term “calciphylaxis” was coined by Hans Selye.

The condition is also described in the literature as metastatic calcinosis cutis and necrotizing or calcifying panniculitis. It is important to note that the presence of renal failure is not an absolute requirement in patients with calciphylaxis, as calciphylaxis has been seen in the absence of renal failure.

Calciphylaxis is a complex disorder with a multifactorial etiology. The exact pathogenesis of calciphylaxis is unclear. Medial calcification and intimal fibrosis of the cutaneous arterioles combined with thrombotic occlusion leading to ischemic skin necrosis is seen in calciphylaxis.

Calciphylaxis is more commonly seen in patients with ESRD, and females are affected more than males. Lower extremities are the most common area involved, with legs being the most common site. Lesions start with tender red areas developing into a livedoid pattern. Solitary or multiple indurated plaques and/or nodules are then seen. Patients may subsequently develop an eschar followed by frank ulceration, gangrene, or sepsis Face and upper extremities are rarely involved. Patients may have palpable deposits of calcium,¹⁶ and bullae may be noted. The most consistent feature of calciphylaxis is pain. Extreme pain is noted when skin around the ulcer is palpated.

There can be significant morbidity and mortality from the disease, most commonly resulting from septicemia due to impaired integrity of the epidermis and dermis.⁴ More than 50 percent of patients die (most commonly from sepsis) within one year of being diagnosed.