Identifying subcutaneous tissue microcalcification by Fluo-3 AM imaging in cutaneous calciphylaxis.

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is typically characterized by subcutaneous tissue calcification and excruciatingly painful cutaneous lesions with high mortality. It is critical for dermatologists to make early diagnosis and appropriate management, yet currently only 56% of calciphylaxis cases are correctly diagnosed by conventional histological stain1. Specially, the identification of subtle calcium deposits of subcutaneous can be challenging but is believed crucial for early diagnosis of calciphylaxis2. More sensitive calcification staining is in high demand. In this study, Fluo-3 AM was found to be a rapid, sensitive and reliable fluorescent probe for the detection of calcium deposits and could be a promising diagnostic tool for calciphylaxis.

Success of small-dose fractionated sodium thiosulfate in the treatment of calciphylaxis in a peritoneal dialysis patient.

BACKGROUND: Calciphylaxis, or calcific uremic arteriolopathy (CUA), is a rare, fatal disorder of microvascular calcification and thrombosis that typically affects patients with end-stage renal disease (ESRD) receiving long-term dialysis. Fewer reports describe calciphylaxis in peritoneal dialysis patients than hemodialysis patients as per a literature review. To date, there are no clear guidelines for CUA diagnosis and treatment. While sodium thiosulfate (STS) has been increasingly used for treatment in recent years, there have also been reports of severe side effects. There is no uniform standard for its usage and dosage, especially for peritoneal dialysis patients. CASE PRESENTATION: We present a case of a 40-year-old Chinese male patient with ESRD on peritoneal dialysis who developed calciphylaxis with severe painful cutaneous ulcers on the fingers and toes that were managed successfully for 6 months with comprehensive treatment composed mainly of small-dose fractionated sodium thiosulfate. CONCLUSIONS: Our experience suggests that the treatment of calciphylaxis requires timely and multi-angle intervention. Treatment with small-dose fractionated sodium thiosulfate has proven effective and tolerated in this patient.

Multicentric Study on High-Frequency Ultrasound Characterization of Calcium Deposits in Dermal and Subcutaneous Calciphylaxis and Calcinosis.

OBJECTIVES: Calcium depositions are frequent in multiple inflammatory dermatosis, they can be explored by ultrasound (US) but the patterns of these depositions have not yet been described. The aim of this study is to describe different patterns of calcium deposition in inflammatory dermatoses. METHODS: The clinical and US data of 58 patients from 7 different centers with inflammatory dermatosis showing ultrasonography-detected calcium depositions was retrospectively reviewed. RESULTS: Dystrophic calcinosis represented 86.2%, calciphylaxis 8.6%, and metastatic calcinosis 5.2%. Three different sonographic patterns of calcium deposition were found: 1) thin hyperechoic bands, parallel to the surface of the epidermis, generating a strong and wide posterior acoustic shadow; 2) hyperechoic spots or lumps with a narrow acoustic shadow; and 3) a linear hyperechoic band parallel to the walls of a blood vessel with also a narrow acoustic shadow. The predominant pattern in metastatic calcifications was type 1, in dystrophic calcifications type 2, and in calciphylaxis type 3. In dystrophic calcinosis, cutis deposits were longer and wider than in calciphylaxis (P < .05). CONCLUSION: New data on inflammatory dermatoses with calcium deposition may be useful for the diagnosis and monitoring of calcium deposits and could avoid the performance of more invasive tests, such as a skin biopsy.

Concomitant calciphylaxis and COVID-19 associated thrombotic retiform purpura.

While initial reports regarding coronavirus disease 2019 (COVID-19) focused on its pulmonary manifestations, more recent literature describes multisystem abnormalities related to its associated microvascular angiopathy. Calciphylaxis is a rare systemic condition characterized by tissue necrosis in the setting of systemic microvascular calcifications. Both COVID-19 and calciphylaxis are procoagulant diagnoses associated with vascular-mediated cutaneous findings. To our knowledge, this is the first report to document the coexistence of COVID-19 associated retiform thrombotic purpura and calciphylaxis in a single patient, to link the pathologic etiologies of the two entities, and to describe the concomitant diagnoses' associated radiologic findings.

Unusual Presentation of Mammary Calciphylaxis in a Patient on Long-Standing Renal Dialysis.

INTRODUCTION: Calciphylaxis is a condition which involves ectopic deposition of calcium in arterioles of various organ systems. Here, we present a rare case of mammographic calcifications in a patient on long-standing renal dialysis. CASE: This is a 57-year-old female who presented to breast clinic with unilateral severe pain and lumpiness of her breast. On mammography, calcifications were identified which were associated with focal lesions on ultrasound. An image-guided core biopsy showed numerous foci of histological calcification within a large area of fat necrosis. The location within arterioles was confirmed by CD31 immunohistochemistry. The diagnosis of fat necrosis with benign calcifications due to mammary calciphylaxis was made. CONCLUSION: The hallmark of calciphylaxis is the deposition of calcium within small- to medium-sized vessel walls. This leads to ischaemia and necrosis of tissue. In the breast, only a few cases were reported in which patients presented with a necrotic lesion resembling carcinoma. These are reviewed in this report.

Management of Penile Calciphylaxis: A Case Study.

BACKGROUND: Calciphylaxis, also called calcific uremic arteriolopathy, is a highly morbid syndrome characterized by calcium deposition and occlusion of small arterial vessels of the dermis and subdermal adipose tissue, leading to necrosis and gangrene. Penile involvement is rare and its management presents considerable challenges. CASE: We review the case of a 47-year-old man with end-stage renal disease managed with hemodialysis, diabetes mellitus, and urinary incontinence who presented with a painful necrotic lesion on his glans penis, and the second and third toes of his right foot. Following diagnosis of calciphylaxis of the toes and penis, he was conservatively managed with topical wound care, sodium thiosulfate adjustment of hemodialysis, and phosphate binder medications. Over the course of 2 months, his wound worsened in the setting of continued urinary incontinence, and before planned diversion with a suprapubic catheter, he progressed to gangrene and sepsis. After a goals-of-care discussion with the patient and family, he elected to forego debridement and was discharged on home-based palliative care. CONCLUSIONS: Penile calciphylaxis is a rare, life-threatening disease that portends a poor prognosis. Conservative principles for management include normalization of calcium phosphate levels and local wound care. Penectomy may not impact survival. Ultimately, each case is individualized, and we encourage establishing goals of care in collaborative discussion with an interdisciplinary care team, patient, and family.

Calciphylaxis: Case Presentation and Review of the Literature.

BACKGROUND: Calciphylaxis is a potentially life-threatening condition involving painful necrotic skin ulcerations, especially of the lower extremities. It is generally associated with chronic kidney failure but may be seen in nonuremic cases. CASE REPORT: We report a case of calciphylaxis in a 60-year-old man with diabetes on dialysis for end-stage renal disease and known to have other typical combination of risk factors associated with calciphylaxis syndrome. On examination, he presented with multiple intensely painful calciphylaxis wounds on his legs and ankles. Despite regular wound management of his condition in our ambulatory care setting, his ulcers deteriorated. The patient's pain also increased and he was referred for hospital admission. CONCLUSION: This case presentation emphasizes the importance of an interdisciplinary team in the treatment of the complex, life-threatening cutaneous manifestations of calciphylaxis. When indicated, prompt referral to a hospital setting is necessary for appropriate care.

Sodium Thiosulfate in the Treatment of Lung and Breast Calciphylaxis: CT and Bone Scintigraphy Findings.

A 19-year-old woman, with normal kidney function, was diagnosed as having visceral calciphylaxis, especially diffuse breast and lung calcification. The calcification findings were clearly shown on CT, and bone scintigraphy revealed diffuse uptake in the thoracic area. Although there was no standard therapeutic approach for this clinical setting, the patient received empiric therapy with sodium thiosulfate. After 5 months of consecutive therapy, the calcification condition had a striking regression.

Calciphylaxis: Comparison of radiologic imaging and histopathology.

BACKGROUND: The current gold standard for diagnosis of calciphylaxis is a skin biopsy specimen demonstrating calcification of small-caliber arteries or arterioles. OBJECTIVE: The aim of this study is to compare diameters of calcified vessels seen in skin biopsy specimens and radiology images of patients with calciphylaxis. METHODS: We conducted a retrospective study of patients with known calciphylaxis from 2009 to 2016 at a community hospital who had both skin biopsy specimens and radiology images taken as part of their routine care. Vascular calcification was compared in skin biopsy specimens and radiology images. RESULTS: Seven patients were identified. Small-vessel calcification as fine as 0.1 to 0.3 mm was identified on plain films in 3 patients; 0.1 to 0.2 mm by mammography in 3 patients, and 0.1 to 0.2 mm by computed tomography imaging in 1 patient, nearly as fine a resolution as on histopathology. LIMITATIONS: This was a single-center study with limited sample size. CONCLUSION: Radiologic imaging might enable more rapid diagnosis of calciphylaxis when skin biopsy specimen is pending or not available.