S3473 Scleredema in a Patient With Autoimmune Pancreatitis: A Rare Clinical Finding

Introduction: IgG4 related disease (IgG4-RD) is a systemic, pro-fibrotic condition that involves infiltration of IgG4+ plasma cells into organs with resultant masses. IgG4-RD skin manifestations are characterized by lymphoplasmacytic infiltrates and include cutaneous plasmacytosis and angiolymphoid hyperplasia among others. We present the case of scleredema, a rare skin manifestation in a patient diagnosed with autoimmune pancreatitis. Case Description/Methods: A 72-year-old man with sole history of prostate cancer treated with radiation presented to the emergency department with abdominal pain, headaches and nausea of 2-week duration. A diagnosis of acute pancreatitis was established, based on clinical presentation, lipase elevation of 483 U/L (13-60 U/L) and acute interstitial edematous pancreatitis with enlargement of the gland (sausage-like swelling) on computed tomography scan. Further workup revealed an elevated serum IgG4 of 120.18 mg/dL (4-86 mg/dL), an IgG4/IgG index >10% and an enlarged pancreas on repeat imaging. Etiologies other than autoimmune disease were excluded. The patient remained asymptomatic following his presentation. Surveillance imaging showed a persistently swollen pancreas with mild diffuse peripancreatic edema. A year following the diagnosis of autoimmune pancreatitis, the patient developed firm, ill-defined, non-tender, non-pruritic subcutaneous nodules on both sides of the upper back. Pathology from punch biopsy revealed lichenoid, perivascular and periadnexal infiltrates of mostly CD3+ and CD4+ lymphocytes, and fibrotic papillary dermal collagen consistent with dermal mucinosis (scleromyxedema). Although the patient remained asymptomatic without recurrent acute pancreatitis episodes, a 12-week course of oral steroid therapy was proposed for the fibrotic skin rash. The course was not completed due to patient reluctance to array of adverse effects. Discussion: Scleredema, which is characterized by glycosaminoglycans and collagen deposition in the dermis, is associated with paraproteinemia and long-standing diabetes mellitus but is rare in association with IgG4-RD. The patient did not have diabetes or other systemic conditions like multiple myeloma. The rash was deemed idiopathic by dermatology. While steroid and anti-CD19 monoclonal antibody therapies may be used for the systemic manifestations of IgG4-RD, it is unknown whether they would provide any benefits for IgG4-related scleredema. Intralesional steroids may be tried for the treatment of these fibrotic skin lesions.