Abstract | March 24, 2024
Gangrenous Calciphylaxis in a Peritoneal Dialysis Patient: Case Report
Learning Objectives
- Identify the clinical manifestations of calciphylaxis in peritoneal dialysis patients, and discuss the therapeutic role of angioplasty in treating concomitant gangrenous calciphylaxis and peripheral arterial disease
Introduction: Calciphylaxis, a rare condition primarily associated with end-stage renal disease (ESRD), manifests as painful cutaneous ulcerations, microvascular calcification, and tissue necrosis. While frequently studied in hemodialysis patients, its presentation and management in peritoneal dialysis (PD) patients remain underexplored. This case describes a patient on PD who developed gangrenous calciphylaxis in the setting of pre-existing peripheral arterial disease, eventually requiring multi-limb amputation despite aggressive, multimodal treatment.
Case Presentation: A 63-year-old male with a history of hypertension, diabetes mellitus type II, ESRD, hyperparathyroidism, and hyperphosphatemia on PD presented with bilateral calf ulcers and penile and toe gangrene. The patient had noticed tender erythematous nodules on his lower legs three months prior, with later development of ulceration and necrosis on the penile glans and toes. Physical exam revealed black necrotic skin on the penile glans, toes, and multiple subcutaneous, firm, erythematous nodules with central necrosis on the legs. His right extremity exhibited weak dorsalis pedis and popliteal pulses. Calciphylaxis was strongly suspected due to the clinical features, ESRD history, hyperparathyroidism, and hyperphosphatemia. Other differential diagnoses were initially considered, such as cellulitis and peripheral arterial disease. Punch biopsy of a subcutaneous nodule confirmed the diagnosis of calciphylaxis, and a right leg angiogram additionally revealed severe peripheral arterial disease at the ankle and below.
Final/Working Diagnosis: Calciphylaxis with co-existing peripheral arterial disease
Management/Outcome: The patient was transitioned to hemodialysis for improved serum phosphorus control and to facilitate treatment with sodium thiosulfate. He received angioplasty of the right posterior tibial artery for treatment of his co-existing peripheral arterial disease. Despite this multimodal treatment, he eventually required multi-limb amputation, including a right and left transmetatarsal amputation.
This case presentation underscores the distinctive clinical presentation of calciphylaxis with co-existing peripheral arterial disease in PD patients. The significance of angioplasty in patients with concomitant calciphylaxis and peripheral arterial disease remains an area requiring further investigation. Further research is essential to optimize the management of this condition in PD populations.
References and Resources
- Gallo Marin B, Aghagoli G, Hu SL, Massoud CM, Robinson-Bostom L. Calciphylaxis and kidney disease: a review. American Journal of Kidney Diseases. 2023;81(2):232-239. doi:10.1053/j.ajkd.2022.06.011.
- Chang JJ. Calciphylaxis: diagnosis, pathogenesis, and treatment. Adv Skin Wound Care. 2019;32(5):205-215. doi:10.1097/01.ASW.0000554443.14002.13.
- Nigwekar SU, Kroshinsky D, Nazarian RM, et al. Calciphylaxis: risk factors, diagnosis, and treatment. American Journal of Kidney Diseases. 2015;66(1):133-146. doi:10.1053/j.ajkd.2015.01.034.
- Jeong HS, Dominguez AR. Calciphylaxis: controversies in pathogenesis, diagnosis and treatment. The American Journal of the Medical Sciences. 2016;351(2):217-227. doi:10.1016/j.amjms.2015.11.015.
- Weenig RH. Pathogenesis of calciphylaxis: Hans Selye to nuclear factor κ-B. Journal of the American Academy of Dermatology. 2008;58(3):458-471. doi:10.1016/j.jaad.2007.12.006.
- Sowers KM, Hayden MR. Calcific uremic arteriolopathy: pathophysiology, reactive oxygen species and therapeutic approaches. Oxidative Medicine and Cellular Longevity. 2010;3(2):109-121. doi:10.4161/oxim.3.2.11354.
- New N, Mohandas J, John GT, et al. Calcific uremic arteriolopathy in peritoneal dialysis populations. International Journal of Nephrology. 2011;2011:1-9. doi:10.4061/2011/982854.
- Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: Risk factors, outcome and therapy. Kidney International. 2002;61(6):2210-2217. doi:10.1046/j.1523-1755.2002.00375.x.
- Gossett C, Suppadungsuk S, Krisanapan P, et al. Sodium thiosulfate for calciphylaxis treatment in patients on peritoneal dialysis: a systematic review. Medicina. 2023;59(7):1306. doi:10.3390/medicina59071306.
- Zhang Y, Corapi K, Luongo M, Thadhani R, Nigwekar S. Calciphylaxis in peritoneal dialysis patients: a single center cohort study. International Journal of Nephrology and Renovascular Disease. 9. doi:https://doi.org/10.2147/IJNRD.S115701.
- Ghosh T, Winchester DS, Davis MDP, el‐Azhary R, Comfere NI. Early clinical presentations and progression of calciphylaxis. Int J Dermatology. 2017;56(8):856-861. doi:10.1111/ijd.13622.
- Rick J, Strowd L, Pasieka HB, et al. Calciphylaxis: part i. Diagnosis and pathology. Journal of the American Academy of Dermatology. 2022;86(5):973-982. doi:10.1016/j.jaad.2021.10.064.
- Barbera V, Di Lullo L, Gorini A, et al. Penile calciphylaxis in end stage renal disease. Case Reports in Urology. 2013;2013:1-3. doi:10.1155/2013/968916.
- Rick J, Rrapi R, Chand S, et al. Calciphylaxis: treatment and outlook—cme part ii. Journal of the American Academy of Dermatology. 2022;86(5):985-992. doi:10.1016/j.jaad.2021.10.063.
- Seethapathy H, Noureddine L. Calciphylaxis: approach to diagnosis and management. Advances in Chronic Kidney Disease. 2019;26(6):484-490. doi:10.1053/j.ackd.2019.09.005.