Intraductal Papillary Mucinous Neoplasm of the Pancreas Arising in a Patient With Limited Cutaneous Systemic Sclerosis

Systemic sclerosis (SS) is a heterogenous autoimmune disease that manifests itself with skin and internal organ involvement. The association of SS and malignancy is an emerging field of study with limited data in the literature. This report highlights the unique case of a patient with limited cutaneous SS (lcSS) found to have an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. In this report, we review the clinical manifestations, serologic findings, and phenotypes of SS. Furthermore, an evaluation of the risk of pancreatic neoplasms in patients with SS will be discussed, as well as the correlation of cancers among SS phenotypes and auto-antibodies. As part of our research, a PubMed search of the following terms was performed: “systemic sclerosis, scleroderma, limited cutaneous systemic sclerosis, CREST syndrome, Raynaud syndrome, cancer, malignancy, pancreas, and intraductal papillary mucinous neoplasm".