Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience

Abstract

Calciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA), there are reported cases of nonuremic calciphylaxis (NUC), which often remain undiagnosed. We conducted a retrospective chart review at our dermatological hospital and evaluated data concerning the epidemiology, comorbidities, medication, laboratory abnormalities, and therapeutic approaches of 60 patients diagnosed with calciphylaxis between 01/2012 and 12/2022. We identified 21 patients diagnosed with NUC and 39 with kidney disease. The predilection sites of skin lesions were the lower legs in 88% (n = 53), followed by the thigh and gluteal regions in 7% (n = 4). Significant differences were identified in comorbidities, such as atrial fibrillation (p < 0.001) and hyperparathyroidism (p < 0.01) accounting for CUA patients. Medication with vitamin K antagonists (p < 0.001), phosphate binders (p < 0.001), and loop diuretics (p < 0.01) was found to be associated with the onset of calciphylaxis. Hyperphosphatemia (p < 0.001), increased parathyroid hormone (p < 0.01) and triglyceride levels (p < 0.01), hypoalbuminemia (p < 0.01) and decreased hemoglobin values (p < 0.001) in the CUA cohort were significantly different from those in the NUC group. All patients with CUA received systemic medication. In contrast, only 38% (n = 8) of patients with NUC received systemic treatment. Striking discrepancies in the treatment of both cohorts were detected. In particular, NUC remains a disease pattern that is still poorly understood and differs from CUA in several important parameters.

Keywords:

• Calciphylaxis
• calcific uremic arteriolopathy
• nonuremic calciphylaxis
• leg ulcer

Acknowledgements

The present work was performed in partial fulfillment of the requirements for obtaining the degree ‘Dr. med.’ of Ms. Sabine Yousuf.

We thank Thomas Breakell for the critical linguistic review of the manuscript.

Authors’ contribution

Ms. Yousuf performed data acquisition, evaluation and provided the first manuscript draft. The present work was performed in partial fulfillment of the requirements for obtaining the degree „Dr. med. ‘at the Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU).

Dorothee Busch assisted with statistics and data evaluation.

Regina Renner evaluated the manuscript draft and commented on the data interpretation.

Stefan Schliep performed dermatohistopathological validation.

Cornelia Erfurt-Berge provided the idea for the project, supervised data analysis, and the first draft of the manuscript.

All authors added their input to the final manuscript and agree with submission of the final version.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Data availability statement

The abstract contains 237 words, and the major text of the article contains 3261 words, three tables, three figures, and 61 references.

Additional information

Funding

This work was not supported by any funding sources and has not been previously presented.