A Fatal Case of Systemic Calciphylaxis in the Gastrointestinal Tract: A Case Report and Literature Review

Abstract

Calciphylaxis is an infrequent yet lethal disease often associated with end-stage kidney disease (ESKD). The most common sites include proximal and distal extremities and the trunk, with few reported in the penis and very few as gastrointestinal (GI) disease. We report a case of systemic calciphylaxis in a middle-aged male, presenting with a colostomy leak and parastomal abscess. Workup revealed severe calcification of the intestinal arteries and ischemic colon necrosis. The patient underwent colectomy, antibiotic therapy, regular hemodialysis (HD), and sodium thiosulphate infusion with clinical stability. Histopathology of the colon revealed ischemic necrosis and pericolonic vessel calcification suggestive of calciphylaxis. It is an important differential to be considered in patients with risk factors presenting with symptoms of gastrointestinal hemorrhage and necrosis with perforation.

Keywords: bowel ischemia; end-stage renal disease (esrd); gastrointestinal calciphylaxis; penile calciphylaxis; tertiary hyperparathyroidism.

Figure 1. Physical examination findings

A and B: Dry gangrene of the digits and right foot with black eschar. C: Post-penectomy wound (red arrow). D: Abdomen showing laparoscopic scar and leak (black arrow) around the colostomy bag. E: Stage IV infected sacral decubitus ulcer.

Figure 2. Radiological findings

A and B: Left and right foot X-rays showing calcification of blood vessels (red arrows) and capillaries with reticular pattern within the soft tissue (blue arrow). C: CT scan of the abdomen and pelvis (axial) showing gas in the soft tissue (red arrow) at the site of colostomy lip. D and E: CT of the abdomen and pelvis (coronal) showing abdominal vessel calcification (red arrow), pelvis vessel calcification (yellow arrow), penile vessel calcification (blue arrow), and lead pipe calcification of the inferior mesenteric artery (black arrow). CT: computed tomography

Figure 3. Histopathology findings on H&E stain of the resected colon

A: Ulceration of the colon epithelium with complete loss of architecture indicating ischemic necrosis (black arrow) with adjacent ongoing ischemic damage with some remnant mucosal gland (blue arrow) (40× magnification). B and C: Extensive basophilic calcification of the blood vessels in the peri-colonic tissue with the absence of inflammatory cell infiltrates in the surrounding tissue (black arrows) (40× and 100× magnification, respectively). D: Fat necrosis and adjacent tissue calcification in the peri-colonic tissue (red arrow) (100× magnification). H&E: hematoxylin and eosin