PENILE CALCIPHYLAXIS C Rathore MD 1 , C Bruins, MD 1 , S Rimmer BS 2 , A Loghmani DO 1 , J Larrazolo MD 1 , S Walvekar MD 1
1 Department of Internal Medicine, LSU Health, New Orleans, LA 2 School of Medicine, LSU Health Sciences Center, New Orleans, LA
INTRODUCTION: Calciphylaxis, calcific uremic arteriolopathy, is a rare skin disorder that presents as intensely painful areas of skin ischemia and necrosis and carries a high morbidity and mortality. The skin lesions of calciphylaxis normally involve adipose-rich areas such as abdomen and thighs, though in much rarer cases have involved the penis. Calciphylaxis is most commonly associated with end-stage renal disease (ESRD). Other associated risk factors include hyperphosphatemia, hypercoagulable states, long-term dialysis use, and warfarin use. CASE: A 50-year-old man with ESRD on HD and mechanical mitral valve replacement on warfarin presented with one month of progressive penile pain and swelling, which worsened to an ulcerated lesion at the glans penis and phimosis. CT scan of the pelvis demonstrated marked diffuse atherosclerotic disease with wall calcification of all major vessels, as well as questionable mild soft tissue edema and skin thickening of the penis. Infectious workup was negative. Urology took the patient to the operating room for circumcision and penile biopsy given concern for malignancy. Surgical pathology results revealed necrotic ulcer with thrombosis and intravascular calcification at the ulcer base. Dermatology felt the biopsy was most consistent with calciphylaxis. The treatment plan was coordinated with nephrology to begin sodium thiosulfate with dialysis. The patient continued to complain of intense pain despite pain medications with palliative care and wound care input. The patient was offered penectomy given refractory pain, however he declined. Although it is recommended to discontinue warfarin in the setting of calciphylaxis as it can worsen skin necrosis, the decision was made to continue warfarin given the presence of his mechanical valve and limited options with ESRD. DISCUSSION: Penile calciphylaxis is particularly rare because of the rich vascular network in the area. Microvascular calcification leads to a cascade of vascular endothelial injury and consequent narrowing and thrombosis of the vessels, which ultimately leads to tissue necrosis from reduced blood flow. There is no approved treatment for calciphylaxis. A collaborative approach involving nephrology, dermatology, wound care, and palliative care is necessary to try and reduce risk factors.
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