Penile calciphylaxis: a case report and literature review
DOI:
https://doi.org/10.18203/2320-6012.ijrms20252166Keywords:
Calciphylaxis, Penile injuries, Haemodialysis, Hyperparathyroidism, NephropathyAbstract
Calciphylaxis is a rare and severe vascular disorder characterized by calcification of small vessels, leading to tissue ischemia and necrosis. It predominantly affects patients with end-stage renal disease (ESRD) and is associated with high mortality. This case report describes a 54-year-old male with ESRD on haemodialysis, diabetes mellitus, hypertension, and secondary hyperparathyroidism, who presented with intense penile pain and whitish discoloration of the glans. Despite initial supportive measures, the patient progressed to wet necrosis of the penis and prepuce, accompanied by acute testicular pain and signs of incipient necrotizing fasciitis. Empirical antibiotic therapy and analgesia were initiated, but the condition worsened, necessitating total penectomy with cystostomy due to extensive necrosis. Postoperative complications included surgical site infection, gastric ulcers, and acral necrosis, culminating in refractory septic shock and multiorgan failure, leading to death. In conjunction with this case, we review the existing literature to contextualize its findings, summarize diagnostic and therapeutic challenges, and highlight gaps in evidence. This case underscores the aggressive nature of penile calciphylaxis, its diagnostic dilemmas, and the limited therapeutic options available. Early recognition and multidisciplinary management are crucial, though prognosis remains poor.
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References
Chiriac A, Grosu OM, Terinte C, Perţea M. Calcific uremic arteriolopathy (calciphylaxis) calls into question the validity of guidelines of diagnosis and treatment. J Dermatolog Treat. 2019;31(5):545-8. DOI: https://doi.org/10.1080/09546634.2019.1618435
Cucchiari D, Torregrosa JV. Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal. Nefrologia. 2018;38(6):579-86. DOI: https://doi.org/10.1016/j.nefroe.2018.09.001
García-Lozano JA, Ocampo-Candiani J, Martínez-Cabriales SA, Garza-Rodríguez V. An Update on Calciphylaxis. Am J Clin Dermatol. 2018;19(4):599-608. DOI: https://doi.org/10.1007/s40257-018-0361-x
Gómez de la Fuente E, Vicente FJ, Álvarez JG, Naz E, Palencia SI, Pinedo F, et al. Calcifilaxis en pacientes dializados. Actas Dermosifiliogr. 2004;95(3):178-82. DOI: https://doi.org/10.1016/S0001-7310(04)76792-7
Hayashi M. Calciphylaxis: diagnosis and clinical features. Clin Exp Nephrol. 2013;17(4):498-503. DOI: https://doi.org/10.1007/s10157-013-0782-z
Munar MA, Alarcón A, Bernabéu R, Morey Gascó J, Losada P, Antón E, et al. A propósito de un caso de lesiones cutáneas y calcifilaxis. Nefrologia. 2001;21(5):501-4.
Polaina Rusillo M, Sánchez Martos MD, Biechy Baldan MM, Liebana Cañada A. Calcifilaxis. Semin Fund Esp Reumatol. 2009;10(4):124-7. DOI: https://doi.org/10.1016/j.semreu.2009.07.001
Verdalles Guzmán U, De La Cueva P, Verde E, García de Vinuesa S, Goicoechea M, Mosse A, et al. Calcifilaxis: complicación grave del síndrome cardio-metabólico en pacientes con Enfermedad Renal Crónica Terminal (ERCT). Nefrologia. 2008;28(1):32-6.
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. DOI: https://doi.org/10.1053/j.ajkd.2015.01.034
Rodríguez-Villarreal I, Callejas R, Sánchez M, Laso N, Gallar P, Ortega O, et al. Calcifilaxis o arteriolopatía urémica calcificante: combinación de tratamientos. Nefroplus. 2010;3(3):39-44.
Jeong HS, Dominguez AR. Calciphylaxis: Controversies in Pathogenesis, Diagnosis and Treatment. Am J Med Sci. 2016;351(2):217-27. DOI: https://doi.org/10.1016/j.amjms.2015.11.015
Rodríguez Weber FL, Cano Aguilar LE. Calcifilaxis. Acta Med Grupo Angeles. 2020;18(1):50-60. DOI: https://doi.org/10.35366/92000
Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med. 2018;378(18):1704-14. DOI: https://doi.org/10.1056/NEJMra1505292
Wu J, Chen L, Dang F, Zha P, Li R, Ran X. Refractory wounds induced by normal-renal calciphylaxis: An under-recognised calcific arteriolopathy. Int Wound J. 2022;20(4):1262-75. DOI: https://doi.org/10.1111/iwj.13951
Zhou Y, Chen Y, Yin G, Xie Q. Calciphylaxis and its co-occurrence with connective tissue diseases. Int Wound J. 2022;20(4):1316-27. DOI: https://doi.org/10.1111/iwj.13972
Jiménez-Gallo D, Ossorio-García L, Linares-Barrios M. Calcinosis Cutis and Calciphylaxis. Actas Dermosifiliogr. 2015;106(10):785-94. DOI: https://doi.org/10.1016/j.adengl.2015.10.018
García Morúa A, Gutiérrez García JD, Arrambide Gutiérrez JG, Gómez Guerra LS. Calcifilaxis de pene: nuestra experiencia en 5 años y revisión de la literatura científica. Actas Urol Esp. 2009;33(9):984-90. DOI: https://doi.org/10.4321/S0210-48062009000900015
Kodumudi V, Jeha GM, Mydlo N, Kaye AD. Management of Cutaneous Calciphylaxis. Adv Ther. 2020;37(12):4797-807. DOI: https://doi.org/10.1007/s12325-020-01504-w
Rrapi R, Chand S, Gabel C, Ko L, Moore KJ, Steele D, et al. Early diagnosis and intervention of calciphylaxis leading to rapid resolution. JAAD Case Rep. 2021;13:65-70. DOI: https://doi.org/10.1016/j.jdcr.2021.04.010
Karpman E, Das S, Kurzrock EA. Penile Calciphylaxis: Analysis of Risk Factors and Mortality. J Urol. 2003;169(6):2206-9. DOI: https://doi.org/10.1097/01.ju.0000064334.85656.a1
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. 2018;13(1):158-63. DOI: https://doi.org/10.1515/med-2018-0025
Torre AC, Bastard DP, Diehl M, Rosa-Diez G, Volonteri V, Mazzuoccolo LD, et al. Calcifilaxis, factores de riesgo, tratamiento y evolución estudio retrospectivo de 39 pacientes. Medicina (B Aires). 2023;84:196-205.
El-Taji O, Bondad J, Faruqui S, Bycroft J. Penile calciphylaxis: a conservative approach. Ann R Coll Surg Engl. 2020;102(2):36-8. DOI: https://doi.org/10.1308/rcsann.2019.0119
Gabel C, Chakrala T, Shah R, Danesh MJ, Dobry AS, Garza-Mayers AC, et al. Penile calciphylaxis: A retrospective case-control study. J Am Acad Dermatol. 2021;85(5):1209-17. DOI: https://doi.org/10.1016/j.jaad.2020.05.042
Wipattanakitcharoen A, Takkavatakarn K, Susantitaphong P. Risk factors, treatment modalities, and clinical outcomes of penile calciphylaxis: systematic review. World J Urol. 2023;41(11):2959-66. DOI: https://doi.org/10.1007/s00345-023-04611-9
Zillioux JM, Geisenhoff A, Gray M. Management of Penile Calciphylaxis. J Wound Ostomy Continence Nurs. 2018;45(6):536-9. DOI: https://doi.org/10.1097/WON.0000000000000483
Wen W, Portales-Castillo I, Seethapathy R, Durant O, Mengesha B, Krinsky S, et al. Intravenous Sodium Thiosulphate for Calciphylaxis of Chronic Kidney Disease. JAMA Netw Open. 2023;6(4):2310068. DOI: https://doi.org/10.1001/jamanetworkopen.2023.10068
Cimmino CB, Costabile RA. Biopsy Is Contraindicated in the Management of Penile Calciphylaxis. J Sex Med. 2014;11(10):2611-7. DOI: https://doi.org/10.1111/jsm.12390