%0 Case Reports
%T Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report.
%A Yan X
%A Jin J
%J Medicine (Baltimore)
%V 97
%N 8
%D Feb 2018
%M 29465536
%F 1.817
%R 10.1097/MD.0000000000010004
%X <B>BACKGROUND: </B>Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature.<BR><B>UNASSIGNED: </B>A 50-year-old woman presented with progressive abnormal liver enzyme levels and was referred to our department.<BR><B>UNASSIGNED: </B>Due to the patient's symptoms, laboratory test results, radiographic findings, and pathologic results, she was diagnosed with PCA associated with AIH-PBC overlap syndrome and SS.<BR><B>METHODS: </B>She was subsequently treated with a combination of ursodeoxycholic acid (UDCA), prednisone, and azathioprine.<BR><B>RESULTS: </B>While this treatment can achieve therapeutic success, it cannot prevent complications from cirrhosis. This patient remains alive but experienced an emergent gastrointestinal hemorrhage.<BR><B>CONCLUSIONS: </B>While we acknowledge that this is a single case, these findings extend our knowledge of immunological diseases associated with PCA and suggest a common, immune-mediated pathogenic pathway between PCA, AIH-PBC overlap syndrome, and SS. After 12 years of follow up, clinical manifestations have developed, and these autoimmune diseases have progressed. The combination of UDCA, prednisone, and azathioprine can achieve therapeutic success but cannot prevent disease progression. Routine follow up for this patient is necessary to document disease progression.