Adult-onset asthma and periocular xanthogranulomas in a patient with lymphoplasmacytic sclerosing pancreatitis.

The clinical syndrome of adult-onset asthma and periocular xanthogranulomas represents a rare systemic autoimmune disorder that has not been previously associated with autoimmune pancreatitis. Herein, we describe the case report of a 61-year-old man with the unique clinical association of adult-onset asthma, atopy, and periocular xanthogranulomas, who had previously been diagnosed with lymphoplasmacytic sclerosing pancreatitis. We have previously reported the case of a patient with lymphoplasmacytic sclerosing pancreatitis that developed postoperative inflammatory pseudotumor of the orbit. These unique, extrapancreatic, ocular manifestations of this disease add to the published observations that it is a systemic disorder with the potential for multiorgan involvement that may have a spectrum of clinical manifestations that includes adult-onset asthma and periocular xanthogranulomas.