Calciphylaxis and Skin Ulcers

Abstract and Introduction

Abstract

Introduction: Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare and severe disorder that presents with skin ischemia and necrosis. Diagnosis is challenging, and even if the condition is diagnosed in the early stages, the mortality rate is exceptionally high, ranging from 45% to 80%.

Case Report: A 55-year-old male with chronic kidney disease secondary to diabetic nephropathy presented with painful, severe, necrotic ulcers in the lower legs and underwent treatment with sodium thiosulfate, debridement of necrotic tissue, and topical oxygen therapy. Complete healing of the ulcers was achieved within 3 months.

Conclusion: This case report raises awareness of this rare condition and details successful treatment in 1 patient.

Introduction

Calciphylaxis is a rare and severe disorder that was first described by Selye^[1] in 1962. It is characterized histologically by calcification of arterioles and capillaries in the dermis and subcutaneous adipose tissue, which results in intensely painful and ischemic skin lesions in areas with increased adiposity, such as the lower extremities and trunk.^[2–4]

Calciphylaxis most commonly affects patients with end-stage renal disease, with an estimated incidence of 1% annually in up to 4% of patients undergoing dialysis, especially females and patients with obesity or diabetes.^[2,5,6] Even if the condition is diagnosed in the early stages, the mortality rate is exceptionally high, reaching up to 80%.^[6]

The rarity of calciphylaxis in addition to the challenging diagnosis and treatment course contribute to the lack of evidence-based clinical practice guidelines for this condition to date. In addition, only a few case reports have been published.^[7]

The current case report discusses the successful treatment of calciphylaxis in 1 patient.