SAEM Clinical Images Series: Penile Lesion with a Poor Prognosis

A 49-year-old male presented to the emergency department of an academic hospital with the chief concern of penile pain. Prior medical history is notable for end-stage renal disease on hemodialysis three times per week, but the patient has not been dialyzed in the past four days, hypertension, insulin-dependent diabetes mellitus, heart failure with reduced ejection fraction (59% two months prior), seizure disorder, prior pulmonary embolism (eight months prior), a left nephrectomy seven months ago due to a gunshot wound, bradycardia status post permanent pacemaker, duodenal ulcer, and dry gangrene of the left foot. The patient was recently admitted to the hospital eight days prior to presentation for purulent urethral discharge where he received a course of topical antibiotics and was discharged. The patient stated he had 10/10 sharp penile pain since discharge from the hospital. He followed up with all “appropriate” appointments. He noticed that his penis started becoming discolored. He then noticed the pain start and grow in intensity without radiating anywhere else except the glans. He denies any discharge or pain with urination with 2-3 small volume voids daily but denies urine trapping.

GU: There is erythema, tenderness, and discoloration of the penile gland. There is no crepitus or swelling.

The rest of the physical exam is benign.

K: 6.5

Magnesium: 2.6

BUN: 70

Creatinine: 8.7

Alkaline phosphatase: 1562 (baseline 1069-1409)

Hemoglobin: 8.9 (at baseline)

WBC: 18.7 (monocyte predominance)

Calcinosis is characterized by the presence of precipitated calcium crystals in soft tissue. The condition is divided into 5 subtypes based on etiology and severity: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis (1). Dystrophic calcinosis cutis is caused by local trauma to the tissue, such as by repeated intravenous access, heel sticks, or other procedures, and is present in patients with normal calcium and phosphorus levels (1). Metastatic calcinosis cutis occurs in patients with underlying disorders of calcium or phosphorus metabolism (and therefore abnormal calcium and phosphorus levels) and can also be associated with calcium deposition in blood vessels, lungs, kidneys, and intestines (1). Iatrogenic calcinosis cutis is caused by the extravasation of intravenous calcium gluconate, calcium chloride, or fluids containing phosphorus (1). Idiopathic calcinosis cutis occurs in the absence of underlying causes like metabolic disorders or tissue damage. Calciphylaxis is the most serious subtype, defined as a vasculopathy of small and medium vessels that leads to ischemic necrosis.

Calciphylaxis is also known as uremic gangrene syndrome or calcific uremic arteriolopathy and is most often seen in patients with chronic renal failure, specifically those on hemodialysis. It is associated with extreme pain, infection, and a 60% to 80% mortality rate in the setting of few and frequently ineffective therapeutic options (2,3). Because of the rich vascular network of the penis, penile calciphylaxis is rare and is often associated with a poor prognosis having an overall mortality rate of 64% and an average of 2.5 months to death (4).

Take-Home Points

  • Calciphylaxis is the presence of calcium crystals in soft tissues and is often seen in hemodialysis patients.
  • Penile calciphylaxis is a rare finding that often portends a poor prognosis with a high mortality rate.

  • Nunley JR. Calcinosis cutis. http://emedicine.medscape.com/article/1103137
  • Vedvyas, C., Winterfield, L. S., & Vleugels, R. A. (2012). Calciphylaxis: a systematic review of existing and emerging therapies. Journal of the American Academy of Dermatology, 67(6), e253–e260. https://doi.org/10.1016/j.jaad.2011.06.009
  • Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015 Jul;66(1):133-46. doi: 10.1053/j.ajkd.2015.01.034. Epub 2015 May 7. PMID: 25960299; PMCID: PMC4696752.
  • 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 May 9;13:158-163. doi: 10.1515/med-2018-0025. PMID: 29756051; PMCID: PMC5941707.