JMIR Publications

ABSTRACT

Scleroderma (SSc), sometimes more colloquially known as systemic sclerosis, is a group of autoimmune diseases that principally affects the skin, blood vessels, muscles, and viscera [1]. One of the more well-known subgroups of scleroderma is the limited cutaneous form of the multisystem connective tissue disorder known as CREST syndrome: calcinosis, Raynaud Phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasis [2]. The focus of this paper will be to present a case of a spontaneous colonic bowel perforation in a patient with incomplete features of CREST. Currently, only a few case reports exist documenting this relatively rare, but recognized, complication. A brief explanation and overview of the complicated hospital and its relation to the patient's SSc will be discussed. While much research exists regarding the esophageal complications of scleroderma patients, it is crucial for the clinician to consider the extremely high rates of extra-esophageal GI involvement of the disease. This consideration may compel the emergency physician to have a lower threshold for additional testing and actions such as cross-sectional imaging, lab tests, and specialist consultations. This recommendation is particularly germane in patients who are not being treated for their disease, as was the case with our patient. Additionally, physicians managing SSc patients ensuing to the ED encounter must anticipate the multitude of complications that can occur.