Penile Calciphylaxis – A Rare, Yet Medically Treatable Disease

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Abstract

Calciphylaxis or calcific uremic arteriolopathy (CUA) is an infrequent complication in patients with renal failure. Its manifestations range from ischemia to gangrene especially in areas of adiposity. Penile calciphylaxis is very rarely seen. Treatment can be medical or surgical. Sodium thiosulphate has shown to be an efficient treatment. Here were present a case of 74-year-old male who presented with penile calciphylaxis and was treated successfully with oral sodium thiosulphate.

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Most cited references 19

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Risk factors and mortality associated with calciphylaxis in end-stage renal disease.

Alan C Breen, Sharon K. Davis, D. Gillen … (2001)

We conducted a case control study to determine risk factors and mortality associated with calciphylaxis in end-stage renal disease. Cases of calciphylaxis diagnosed between December 1989 and January 2000 were identified. Three controls were identified for each hemodialysis patient, with calciphylaxis matched to the date of initiation of hemodialysis. Laboratory data and medication doses were recorded during the 12 months prior to the date of diagnosis and at the time of diagnosis of calciphylaxis. Conditional logistic regression was used to identify risk factors for calciphylaxis. Cox proportional hazards models were used to estimate the risk of death associated with calciphylaxis. Nineteen cases and 54 controls were identified. Eighteen patients were hemodialysis patients, and one had a functioning renal allograft. Diagnosis was confirmed by skin biopsy in 16 cases. Women were at a sixfold higher risk of developing calciphylaxis (OR = 6.04, 95% CI 1.62 to 22.6, P = 0.007). There was a 21% lower risk of calciphylaxis associated with each 0.1 g/dL increase in the mean serum albumin during the year prior to diagnosis and at the time of diagnosis of calciphylaxis (OR = 0.79, 95% CI, 0.64 to 0.99, P = 0.037, and OR = 0.80, 95% CI, 0.67 to 0.96, P = 0.019, respectively). There was a 3.51-fold increase in the risk of calciphylaxis associated with each mg/dL increase in the mean serum phosphate during the year prior to diagnosis (95% CI, 0.99 to 12.5, P = 0.052). At the time of diagnosis of calciphylaxis, for each 10 IU/L increment in alkaline phosphatase, the risk of calciphylaxis increased by 19% (OR = 1.19, 95% CI, 1.00 to 1.40, P = 0.045). Body mass index, diabetes, blood pressure, aluminum, and higher dosage of erythropoietin and iron dextran were not independent predictors of calciphylaxis. Calciphylaxis independently increased the risk of death by eightfold (OR = 8.58, 95% CI, 3.26 to 22.6, P < 0.001). Female gender, hyperphosphatemia, high alkaline phosphatase, and low serum albumin are risk factors for calciphylaxis. Calciphylaxis is associated with a very high mortality.

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Penile calciphylaxis: analysis of risk factors and mortality.

Edward Karpman, Satki Das, Eric Kurzrock (2003)

Penile calciphylaxis is a rare condition resulting in infection and gangrene. Most cases are associated with systemic calciphylaxis. The pathophysiology, diagnosis and management of penile calciphylaxis as a distinct entity have received little attention. We reviewed the literature to increase understanding of this disease. A retrospective review of the literature was performed after treating a case of penile calciphylaxis. Patient characteristics, presentation, serum chemistry studies, management and outcomes are reported. A total of 34 cases of penile calciphylaxis were identified in the literature including our patient. Average patient age was 58 years. All patients had end stage renal disease, and diabetes mellitus was a co-morbidity in 76%. Additional areas of gangrene beyond the genitalia were found in two-thirds of patients. Average calcium phosphate product was 78.5 mg.2/dl.2 (range 20.6 to 52.5) and mean parathormone level was 553 pg./ml. (10 to 65). Parathyroidectomy was performed in 8 patients. All patients were treated with either local débridement/wound care or partial/total penectomy. Survival was better in patients who underwent parathyroidectomy (75%) than in those treated with local débridement or penectomy alone (28%). The overall mortality associated with this disease was 64% with a mean time to death of 2.5 months. Penile calciphylaxis is a result of medial calcification and fibrosis of blood vessels. The co-morbidity and mortality associated with this disease are extremely high. Secondary hyperparathyroidism and an increased calcium phosphate are characteristic and require aggressive medical management. Surgical management of penile lesions and parathormone is controversial. Our review suggests that parathyroidectomy may improve survival and that survival is independent of the type of local treatment for the penile lesions.

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Calciphylaxis: a systematic review of existing and emerging therapies.

Chetan Vedvyas, Laura S Winterfield, Ruth Vleugels (2012)

Calciphylaxis, also known as calcific uremic arteriolopathy, is a cutaneous ischemic small vessel vasculopathy seen in 1 to 4% of patients with chronic kidney disease on hemodialysis. It is associated with extreme pain and a 60 to 80% mortality rate in the setting of few and frequently ineffective therapeutic options, although this may be changing based on reports of success with newer therapies.

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Author and article information

Journal

Journal ID (nlm-ta): Indian J Nephrol

Journal ID (iso-abbrev): Indian J Nephrol

Journal ID (publisher-id): IJN

Journal ID (publisher-id): Indian J Nephrol

Title: Indian Journal of Nephrology

Publisher: Wolters Kluwer - Medknow (India )

ISSN (Print): 0971-4065

ISSN (Electronic): 1998-3662

Publication date (Print): Jul-Aug 2023

Publication date (Electronic): 20 February 2023

Volume: 33

Issue: 4

Pages: 300-303

Affiliations

[1] Department of Nephrology, KG Hospital and Post Graduate Institute, Coimbatore, Tamil Nadu, India

Author notes

Address for correspondence: Dr. Sakthi Selva Kumar Sampathkumar, 145/52, Ayyankulam Street, Tiruvannamalai, Tamil Nadu - 606601, India. E-mail: sakthus@ 123456gmail.com

Article

Publisher ID: IJN-33-300

DOI: 10.4103/ijn.ijn_157_21

PMC ID: 10503586

PubMed ID: 37781553

SO-VID: 895f9c6a-a9dd-4ff3-b587-a3c155836b13

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This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

History

Date received : 09 April 2021

Date revision received : 20 September 2021

Date accepted : 19 January 2022

Penile Calciphylaxis – A Rare, Yet Medically Treatable Disease

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Abstract

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Karger: Nephrology

Most cited references 19

Risk factors and mortality associated with calciphylaxis in end-stage renal disease.

Penile calciphylaxis: analysis of risk factors and mortality.

Calciphylaxis: a systematic review of existing and emerging therapies.

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