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Published in: Journal of Medical Case Reports 1/2022

Open Access 01-12-2022 | Case report

Calciphylaxis of the penis and distal digits: a case report

Authors: Gordon Smilnak, Michael Jiang, Bijal Jain

Published in: Journal of Medical Case Reports | Issue 1/2022

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Abstract

Background

Calciphylaxis is a rare, often fatal disease resulting from calcification of dermal arterioles and capillaries. Usually diagnosed in patients with end-stage renal disease, this disorder typically presents as necrotic, nonhealing ulcers in acral or adipose areas. Here we report the case of an elderly man who was found to have calciphylaxis of the distal digits and penis, the latter of which is an uncommon site of disease that carries a particularly poor prognosis.

Case presentation

A 73-year-old African American man with multiple medical comorbidities including dialysis-dependent end-stage renal disease presented with worsening painful, necrotic lesions on his glans penis and several distal digits over the last 2 months. The wound on the glans was foul smelling with overlying purulence and had been unsuccessfully treated with amoxicillin–clavulanic acid. Discovery of diffuse intravascular calcification on computed tomography, in addition to a markedly elevated calcium–phosphate product immediately prior to the onset of his ulcers, led to the diagnosis of calciphylaxis. The patient was initiated on sodium thiosulfate without improvement in his lesions, and he died 3 months later after another prolonged hospitalization.

Conclusions

While calciphylaxis is a rare disease, involvement of the distal digits and especially the penis is even more uncommon and portends a particularly poor prognosis: 6-month mortality rates are reportedly as high as 70%. This suggests that prompt recognition and management of the disease is required; however, despite receiving standard therapy, our patient failed to experience improvement in his disease and instead developed several more fingertip ulcers at blood glucose sample points during his hospitalization. A corollary of the case presented here is the need for more effective management of calciphylaxis, especially for patients in whom uncommon sites, such as the penis, are involved.
Literature
1.
go back to reference Brandenburg VM, Kramann R, Rothe H, Kaesler N, Korbiel J, Specht P, et al. Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. Nephrol Dial Transpl. 2017;32(1):126–32. Brandenburg VM, Kramann R, Rothe H, Kaesler N, Korbiel J, Specht P, et al. Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. Nephrol Dial Transpl. 2017;32(1):126–32.
2.
go back to reference Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med. 2018;378(18):1704–14.CrossRef Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med. 2018;378(18):1704–14.CrossRef
3.
go back to reference Nigwekar SU, Brunelli SM, Meade D, Wang W, Hymes J, Lacson E Jr. Sodium thiosulfate therapy for calcific uremic arteriolopathy. Clin J Am Soc Nephrol. 2013;8(7):1162–70.CrossRef Nigwekar SU, Brunelli SM, Meade D, Wang W, Hymes J, Lacson E Jr. Sodium thiosulfate therapy for calcific uremic arteriolopathy. Clin J Am Soc Nephrol. 2013;8(7):1162–70.CrossRef
4.
go back to reference McCarthy JT, El-Azhary RA, Patzelt MT, Weaver AL, Albright RC, Bridges AD, et al. Survival, risk factors, and effect of treatment in 101 patients with calciphylaxis. Mayo Clin Proc. 2016;91(10):1384–94.CrossRef McCarthy JT, El-Azhary RA, Patzelt MT, Weaver AL, Albright RC, Bridges AD, et al. Survival, risk factors, and effect of treatment in 101 patients with calciphylaxis. Mayo Clin Proc. 2016;91(10):1384–94.CrossRef
5.
go back to reference O’Neil B, Southwick AW. Three cases of penile calciphylaxis: diagnosis, treatment strategies, and the role of sodium thiosulfate. Urology. 2012;80(1):5–8.CrossRef O’Neil B, Southwick AW. Three cases of penile calciphylaxis: diagnosis, treatment strategies, and the role of sodium thiosulfate. Urology. 2012;80(1):5–8.CrossRef
6.
go back to reference Sandhu G, Gini MB, Ranade A, Djebali D, Smith S. Penile calciphylaxis: a life-threatening condition successfully treated with sodium thiosulfate. Am J Ther. 2012;19(1):e66–8.CrossRef Sandhu G, Gini MB, Ranade A, Djebali D, Smith S. Penile calciphylaxis: a life-threatening condition successfully treated with sodium thiosulfate. Am J Ther. 2012;19(1):e66–8.CrossRef
7.
go back to reference Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015;66(1):133–46.CrossRef Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015;66(1):133–46.CrossRef
8.
go back to reference Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol. 2008;3(4):1139–43.CrossRef Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol. 2008;3(4):1139–43.CrossRef
9.
go back to reference Nigwekar SU, Solid CA, Ankers E, Malhotra R, Eggert W, Turchin A, et al. Quantifying a rare disease in administrative data: the example of calciphylaxis. J Gen Intern Med. 2014;29:S724–31.CrossRef Nigwekar SU, Solid CA, Ankers E, Malhotra R, Eggert W, Turchin A, et al. Quantifying a rare disease in administrative data: the example of calciphylaxis. J Gen Intern Med. 2014;29:S724–31.CrossRef
10.
go back to reference Angelis M, Wong LL, Myers SA, Wong LM. Calciphylaxis in patients on hemodialysis: a prevalence study. Surgery. 1997;122(6):1083–9.CrossRef Angelis M, Wong LL, Myers SA, Wong LM. Calciphylaxis in patients on hemodialysis: a prevalence study. Surgery. 1997;122(6):1083–9.CrossRef
11.
go back to reference Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007;56(4):569–79.CrossRef Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007;56(4):569–79.CrossRef
12.
go back to reference Udomkarnjananun S, Kongnatthasate K, Praditpornsilpa K, Eiam-Ong S, Jaber BL, Susantitaphong P. Treatment of calciphylaxis in CKD: a systematic review and meta-analysis. Kidney Int Rep. 2019;4(2):231–44.CrossRef Udomkarnjananun S, Kongnatthasate K, Praditpornsilpa K, Eiam-Ong S, Jaber BL, Susantitaphong P. Treatment of calciphylaxis in CKD: a systematic review and meta-analysis. Kidney Int Rep. 2019;4(2):231–44.CrossRef
13.
go back to reference Yang TY, Wang TY, Chen M, Sun FJ, Chiu AW, Chen YH. Penile calciphylaxis in a patient with end-stage renal disease: a case report and review of the literature. Open Med (Wars). 2018;13:158–63.CrossRef Yang TY, Wang TY, Chen M, Sun FJ, Chiu AW, Chen YH. Penile calciphylaxis in a patient with end-stage renal disease: a case report and review of the literature. Open Med (Wars). 2018;13:158–63.CrossRef
14.
go back to reference Karpman E, Das S, Kurzrock EA. Penile calciphylaxis: analysis of risk factors and mortality. J Urol. 2003;169(6):2206–9.CrossRef Karpman E, Das S, Kurzrock EA. Penile calciphylaxis: analysis of risk factors and mortality. J Urol. 2003;169(6):2206–9.CrossRef
15.
go back to reference Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dial. 2002;15(3):172–86.CrossRef Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dial. 2002;15(3):172–86.CrossRef
16.
go back to reference Ghosh T, Winchester DS, Davis MDP, El-Azhary R, Comfere NI. Early clinical presentations and progression of calciphylaxis. Int J Dermatol. 2017;56(8):856–61.CrossRef Ghosh T, Winchester DS, Davis MDP, El-Azhary R, Comfere NI. Early clinical presentations and progression of calciphylaxis. Int J Dermatol. 2017;56(8):856–61.CrossRef
17.
go back to reference Halasz CL, Munger DP, Frimmer H, Dicorato M, Wainwright S. Calciphylaxis: comparison of radiologic imaging and histopathology. J Am Acad Dermatol. 2017;77(2):241-6.e3.CrossRef Halasz CL, Munger DP, Frimmer H, Dicorato M, Wainwright S. Calciphylaxis: comparison of radiologic imaging and histopathology. J Am Acad Dermatol. 2017;77(2):241-6.e3.CrossRef
18.
go back to reference Chang JJ. Calciphylaxis: diagnosis, pathogenesis, and treatment. Adv Skin Wound Care. 2019;32(5):205–15.CrossRef Chang JJ. Calciphylaxis: diagnosis, pathogenesis, and treatment. Adv Skin Wound Care. 2019;32(5):205–15.CrossRef
19.
go back to reference Cimmino CB, Costabile RA. Biopsy is contraindicated in the management of penile calciphylaxis. J Sex Med. 2014;11(10):2611–7.CrossRef Cimmino CB, Costabile RA. Biopsy is contraindicated in the management of penile calciphylaxis. J Sex Med. 2014;11(10):2611–7.CrossRef
20.
go back to reference Bani-Hani M, Nawafleh S, Al-zubi M, Alkhatatbeh H, Altal Y, Sarhan MY, et al. Penile calciphylaxis diagnosis and treatment challenges a case report. Int J Surg Case Rep. 2020;77:187–90.CrossRef Bani-Hani M, Nawafleh S, Al-zubi M, Alkhatatbeh H, Altal Y, Sarhan MY, et al. Penile calciphylaxis diagnosis and treatment challenges a case report. Int J Surg Case Rep. 2020;77:187–90.CrossRef
21.
go back to reference Baldwin C, Farah M, Leung M, Taylor P, Werb R, Kiaii M, et al. Multi-intervention management of calciphylaxis: a report of 7 cases. Am J Kidney Dis. 2011;58(6):988–91.CrossRef Baldwin C, Farah M, Leung M, Taylor P, Werb R, Kiaii M, et al. Multi-intervention management of calciphylaxis: a report of 7 cases. Am J Kidney Dis. 2011;58(6):988–91.CrossRef
22.
go back to reference Harris C, Kiaii M, Lau W, Farah M. Multi-intervention management of calcific uremic arteriolopathy in 24 patients. Clin Kidney J. 2018;11(5):704–9.CrossRef Harris C, Kiaii M, Lau W, Farah M. Multi-intervention management of calcific uremic arteriolopathy in 24 patients. Clin Kidney J. 2018;11(5):704–9.CrossRef
23.
go back to reference Yu WY, Bhutani T, Kornik R, Pincus LB, Mauro T, Rosenblum MD, et al. Warfarin-associated nonuremic calciphylaxis. JAMA Dermatol. 2017;153(3):309–14.CrossRef Yu WY, Bhutani T, Kornik R, Pincus LB, Mauro T, Rosenblum MD, et al. Warfarin-associated nonuremic calciphylaxis. JAMA Dermatol. 2017;153(3):309–14.CrossRef
24.
go back to reference Adrogué HJ, Frazier MR, Zeluff B, Suki WN. Systemic calciphylaxis revisited. Am J Nephrol. 1981;1(3–4):177–83.PubMed Adrogué HJ, Frazier MR, Zeluff B, Suki WN. Systemic calciphylaxis revisited. Am J Nephrol. 1981;1(3–4):177–83.PubMed
25.
go back to reference Campbell RA, Alzweri LM, Sopko NA, Macura KJ, Burnett AL. Penile calciphylaxis: the use of radiological investigations in the management of a rare and challenging condition. Urol Case Rep. 2017;13:113–6.CrossRef Campbell RA, Alzweri LM, Sopko NA, Macura KJ, Burnett AL. Penile calciphylaxis: the use of radiological investigations in the management of a rare and challenging condition. Urol Case Rep. 2017;13:113–6.CrossRef
26.
go back to reference Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int. 2002;61(6):2210–7.CrossRef Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int. 2002;61(6):2210–7.CrossRef
27.
go back to reference Mazhar AR, Johnson RJ, Gillen D, Stivelman JC, Ryan MJ, Davis CL, et al. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int. 2001;60(1):324–32.CrossRef Mazhar AR, Johnson RJ, Gillen D, Stivelman JC, Ryan MJ, Davis CL, et al. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int. 2001;60(1):324–32.CrossRef
Metadata
Title
Calciphylaxis of the penis and distal digits: a case report
Authors
Gordon Smilnak
Michael Jiang
Bijal Jain
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2022
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-021-03231-4

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