Calciphylaxis, most commonly diagnosed in patients with end-stage renal disease (ESRD) but also affecting patients with normal renal function, is a devastating disease characterized by the development of ischemic skin lesions and associated with substantial patient morbidity and high 1-year mortality rates.1 Calciphylaxis was thought to be a rare disease; however, in recent years, reports and publications on the topic have increased.2 Whether this increase is due to a rise in incidence or improved recognition is unclear. Calciphylaxis is primarily a clinical diagnosis, made by the presence of livedoid skin changes or retiform ulcers, often with a thick eschar, overlying painful indurated subcutaneous plaques located predominantly in adipose-rich areas in patients with risk factors such as chronic kidney disease, obesity, long-term warfarin exposure, autoimmune disease, long-term corticosteroid use, hypercoagulable states, liver disease, and other comorbidities. Formal diagnosis has traditionally necessitated a skin biopsy; however, whether histologic confirmation is needed for typical cases has been debated, because the procedure could predispose the patient to significant morbidity, including inducing an ulcer, poor wound healing, and subsequent infection, and trigger worsening of the thrombotic component of the disease. Avoidance of ulceration is important because ulcers are associated with higher mortality.1
Dominguez AR. New Debates Surrounding the Histopathological Findings of Calciphylaxis—Do These Give Us New Insights Into Pathogenesis of the Disease? JAMA Dermatol. 2019;155(7):773–775. doi:10.1001/jamadermatol.2019.0051
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: