Systemic sclerosis with associated endometrial squamous metaplasia, liver mass, adrenal lesion and bilateral breast masses: Case report

Systemic sclerosis is a rare and chronic autoimmune connective tissue disease that involves widespread vascular dysfunction, as well as fibrosis of the skin and internal organs. With the development of organ-based treatment, including ACE-inhibitors for renal crisis and goal directed therapy for heart failure, the mortality associated with renal and cardiac involvement has decreased significantly. Consequently, the increased risk of malignancy in patients with systemic sclerosis has become a topic of concern. Once the diagnosis of systemic sclerosis is made, various associated disease processes, including malignancy, must be screened for. This case report details a 44 year old female presenting to the Emergency Department with abdominal pain, vaginal bleeding, pruritic rash, and two syncopal episodes. The patient was ultimately diagnosed with systemic sclerosis with extensive “salt and pepper” skin pigmentation and tightening over the arms, chest, and back, with associated bilateral breast masses, an adrenal lesion, liver mass, and endometrial squamous metaplasia. Why should an emergency physician be aware of this? A chief complaint as common as “syncope” can be an atypical presentation of less common, difficult to diagnose conditions in the emergency department, such as systemic sclerosis. While systemic sclerosis is not a diagnosis readily made in the emergency department, prompt recognition and appropriate consulting services or referrals can prevent long-term devastating complications and associated morbidity and mortality.