Development of palliative medicine in the United Kingdom and Ireland
Eduardo Bruera, Irene Higginson, Charles F von Gunten, Tatsuya Morita in Textbook of Palliative Medicine and Supportive Care, 2015
Calciphylaxis is a rare and serious disorder characterized by systemic medial calcification of the arterioles that leads to ischemia and subcutaneous necrosis. Â 85 Calciphylaxis most commonly occurs in patients with end-stage renal disease (ESRD) who are on hemodialysis or who have recently received a renal transplant. Calciphylaxis should be suspected in patients with skin lesions characterized by painful, nonulcerating subcutaneous nodules or plaques, nonhealing ulcers, and/or necrosis, which are most commonly present in the thigh and areas of increased adiposity. Â 86,87 Ulceration carries a mortality of greater than 80%. There should be an aggressive program of wound care and adequate pain control, avoidance of local tissue trauma.
Wound Healing, Ulcers, and Scars
Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang in Roxburgh's Common Skin Diseases, 2022
Clinical presentation: Patients present with painful necrotic ulcerations that can extend into the epidermal, dermal, and subcutaneous adipose tissue layers (Figure 17.6). The pathophysiology is not well understood; however, previous epidemiologic studies have found that calciphylaxis is more prevalent in those who are on dialysis for end-stage renal disease. The incidence of uremic calciphylaxis is relatively low, but it is often rapidly progressive and carries high morbidity and mortality rates.
Endocrine emergencies with skin manifestations
Biju Vasudevan, Rajesh Verma in Dermatological Emergencies, 2019
For calciphylaxis, medical therapy includes bisphosphonates, sodium thiosulfate, antibiotics, hyperbaric oxygen, and aggressive wound care and débridement of necrotic tissue. Total or subtotal parathyroidectomy with autotransplantation may be of therapeutic benefit [17].
Surgical treatment of nonuremic calciphylaxis: a case report and review of literature
Published in Case Reports in Plastic Surgery and Hand Surgery, 2023
Minami Tamagake, Munetomo Nagao, Chieko Miura, Yoshimichi Imai
Calciphylaxis is a rare and extremely painful disease characterized by painful skin ulcerations and necrosis in various areas of the body and has a high mortality rate, with 1-year mortality rate reported as 45–80% [1]. Calciphylaxis is typically diagnosed in patients with end-stage kidney disease, affecting up to 4% of long-term dialysis patients, also known as calcific uremic arteriolopathy (CUA) [1]. Exceptionally, some nonuremic cases of calciphylaxis have also been reported. Although wounds of calciphylaxis are often difficult to heal, effective treatment has not yet been established. Calcification of the medial arterioles in subcutaneous and adipose tissues leads to vascular thromboses, resulting in ischemic necrosis or ulcerations [2]. Despite the high mortality rate, mainly due to sepsis attributed to wound infections [3], aggressive surgical treatment, such as extensive debridement or reconstructive surgery, is uncommon because ischemic tissue is thought to delay healing. On the other hand, leaving infected calciphylaxis lesions can lead to sepsis; therefore, infected or necrotic tissue may require emergent surgical debridement. We report the treatment over 2 years of a patient with nonuremic calciphylaxis caused by trauma, who was successfully treated with aggressive debridement and reconstruction using split-thickness skin grafting.
Leg ulceration with histological features of pseudoxanthoma elasticum
Published in Baylor University Medical Center Proceedings, 2021
Usman Asad, Sheevam Shah, Palak Parekh
Our histological findings of ulceration with mixed inflammation and reactive vascular changes were nonspecific. We wanted to rule out the possibility of calciphylaxis, which presents with findings of intimal and medial calcinosis of vessel walls, ischemic necrosis of the epidermis, vascular thrombosis, and diffuse fat necrosis with a mixed infiltrate of neutrophils, lymphocytes, and histiocytes.3 Calciphylaxis clinically presents as ulceration associated with severe pain, most often on the lower extremities and predominantly in fatty areas, and may herald a poor prognosis, with complications including sepsis and even death. Our biopsy, which showed elastic tissue calcification, was nondiagnostic for calciphylaxis and was suggestive of PXE. PXE presents clinically as yellowish papules coalescing into plaques most commonly on the neck, axilla, flexural areas, and groin and may be associated with angioid streaks of the retina potentially leading to blindness, claudication, gastrointestinal bleeding, angina, and cardiac complications. PXE presents histologically as calcified elastic fibers in the mid to lower dermis with a characteristic “steel wool” appearance. However, our patient had no clinical features of PXE. Lipodermatosclerosis and ulceration in the setting of venous insufficiency and history of renal failure were on the clinical differential.
Risk factors for calciphylaxis in Chinese hemodialysis patients: a matched case-control study
Published in Renal Failure, 2021
Yuqiu Liu, Xiaoliang Zhang, Xiaotong Xie, Xin Yang, Hong Liu, Rining Tang, Bicheng Liu
The medical records of maintenance hemodialysis patients who were newly diagnosed with calciphylaxis at Zhongda Hospital affiliated to Southeast University from October 1, 2017 to December 31, 2018 were retrospectively evaluated. The patients with clinically suspected calciphylaxis based on characteristic skin lesions such as painful purpura and ischemic ulcer required histopathological examination. Typical pathological manifestations were medial calcification and intimal fibroplasia of small arteries, extravascular calcium deposition or thrombosis of pannicular and dermal arterioles. A total of 20 patients were newly diagnosed with calciphylaxis (case group). Subsequently, contemporaneous patients receiving hemodialysis without calciphylaxis were randomly selected as control group. The controls were matched to cases in a 2:1 ratio by age (year, within ±3) and duration of hemodialysis (month, within ±20%) as matching factors. Patients in control group were alive at the time of the survey. The study protocol was approved by the Ethics Committee for Clinical Research of Zhongda Hospital Affiliated to Southeast University (Approval number: 2018ZDSYLL100-P01), and complied with the Declaration of Helsinki. All participants' informed consent was obtained.
Related Knowledge Centers
- Chronic Kidney Disease
- Necrosis
- Ischemia
- Dermis
- Skin
- Thrombosis
- Adipose Tissue
- Cell
- Kidney
- Kidney Dialysis