Objective: Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. 18.4% cases of PNP are associated with Castleman disease. The purpose of our study is to analyze clinical feature, histopathology and ultrasound finding of castleman disease with PNP.
Method: From 1999 to 2008, 12 patients of castleman disease with PNP which were conformed by pathology were studied. Clinical feature, histopathology, ultrasound finding of those patients were analyzed. The pertinent literatures were reviewed.
Result: From 9.0×9.18×7.5cm to 3.0×3.0×3.0cm, 12 lesions were founded by ultrasound examination. 9 lesions were founded in retroperitoneum, and 3 lesions were founded in pelvis. All of 12 cases manifested as well-defined, round or uniform hypoechogenic mass. Calcification was founded in 3 cases. Color Doppler and Power Doppler revealed hypervascularity in all cases.
Conclusion: Ultrasound is valuable techniques in diagnosing Castleman disease with PNP.Castleman disaese should be always suspected when retroperitoneal or pelvic mass is combined with PNP.