INTRODUCTION
Calcific arteriolopathy (CA), also known as calciphylaxis, is a systemic vascular disorder characterized by calcium deposits in the arteriolar tunica media, tunica intima hyperplasia, and endovascular fibrosis, leading to ischemic necrosis of affected tissues.1
According to the associated diseases, it is classified as uremic CA (UCA) and non-uremic CA. The former is the most frequent and affects patients with chronic kidney diseases (CKD), especially those on dialysis.2,3
Skin involvement is characterized by reticular purpuric maculae, which evolve into necrotic ulcers and/or indurated nodules, which are usually accompanied with severe pain. Although the skin is the most frequently affected organ, it can also involve other organs and systems.5,6 Differential diagnosis with other diseases is important.7
Interdisciplinary treatment and all the factors involved in the development of symptoms must be considered.6 Treatment should be based on three pillars: local wound management, correction of the different predisposing factors, and the use of agents that inhibit the calcification process.6
According to the associated diseases, it is classified as uremic CA (UCA) and non-uremic CA. The former is the most frequent and affects patients with chronic kidney diseases (CKD), especially those on dialysis.2,3
Recent studies suggest that the key to the physiopathology of its development is the transformation of the smooth muscle cells of the tunica media of the dermal and hypodermal arterioles into osteoblasts and the imbalance between the factors that promote and inhibit vascular calcification.3,4 Elevated serum phosphorus leads to a phenotype switch from adipocyte to osteoblast-like cells that favor calcification of the arteriolar tunica media.5 However, CA physiopathology is complex and multifactorial.1-4
Skin involvement is characterized by reticular purpuric maculae, which evolve into necrotic ulcers and/or indurated nodules, which are usually accompanied with severe pain. Although the skin is the most frequently affected organ, it can also involve other organs and systems.5,6 Differential diagnosis with other diseases is important.7
CA is unusual and has a high morbidity and mortality. Patients with CKD have a worse prognosis, with a mortality of 45% to 80% at 12 months versus 25% to 45% in patients without CKD.6 Patients with central location of lesions, high body mass index, and ulcerated lesions are at increased risk of death caused by sepsis secondary to superinfection of the wounds.6
Interdisciplinary treatment and all the factors involved in the development of symptoms must be considered.6 Treatment should be based on three pillars: local wound management, correction of the different predisposing factors, and the use of agents that inhibit the calcification process.6
CASE REPORT
We present a 42-year-old female patient with systemic lupus erythematosus diagnosed at age 20, CKD, and kidney transplant, with subsequent graft loss, on non-automated peritoneal dialysis (PD). She was referred from another institution due to severe hypocalcemia, bone pain and rapidly progressing skin lesions that began in the late postoperative period of partial parathyroidectomy due to severe hyperparathyroidism (intact PTH 3600 pg/dL).
Physical examination showed multiple 10/10 painful erythematous nodules and ulcers of variable sizes, irregular in shape, with erythematous-purpuric edges, a necrotic background, located to the right hypochondriac region and the thighs. (Figure 1) Reticular purpuric maculae were observed
