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Abstract
We present the case of a 57-year-old man with known cutaneous manifestations of malignant atrophic papulosis, also known as Köhlmeier-Degos disease, who developed an almost-fatal small bowel perforation following a parathyroidectomy. He required two surgical interventions during his initial hospitalization and was started on eculizumab. Despite these therapies, the patient developed recurrent bowel perforations and ultimately died.