Calciphylaxis

Calciphylaxis, or calcific ureamic arteriolopathy, is a rare condition which manifests as subcutaneous vascular calcification and cutaneous necrosis (small blood vessels of the fat tissue and the skin). Some authors describe as a syndrome of vascular calcification, thrombosis and skin necrosis.

Epidemiology

It is seen most often in patients with end stage renal disease although (usually on haemodialysis) although this is not an absolute requirement. There may be a greater female predilection.

Clinical presentation

The cutaneous 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.

Patients may have palpable deposits of calcium, and bullae may be noted. The most consistent feature of calciphylaxis is pain. Extreme pain is noted when the skin around the ulcer is palpated.

Pathology

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

Distribution

The lower extremities are the most common area to be involved, with legs being the most common site while the face and upper extremities are rarely involved.

Treatment and prognosis

There can be significant morbidity and mortality from the disease, most commonly resulting from septicaemia due to impaired integrity of the epidermis and dermis.

More than 50 percent of patients die (most commonly from sepsis) within one year of being diagnosed.

History and etymology

The condition was first described in 1898 by Bryant and White although the term “calciphylaxis” was coined by Hans Selye in 1962.