Successful treatment of Kimura disease with Mycophenolate Mofetil: a report of two cases and review of the literature.

Kimura Disease (KD) is a rare, chronic inflammatory disease that manifests as subcutaneous tissue swelling, often accompanied by lymphadenopathy, peripheral eosinophilia, and elevated serum IgE levels. Reported treatment options include surgery, systemic corticosteroids, and immunosuppressives. We present two patients with symptomatic KD successfully treated with Mycophenolate Mofetil (MMF). The first case is a 65-year-old Chinese man who presented with erythematous papular lesions on the neck and arms, severe Raynaud’s phenomenon, cervical lymphadenopathy, and peripheral eosinophilia. He was treated with a combination of MMF and prednisone, with resolution of his skin lesions, lymphadenopathy, and eosinophilia, and he was able to decrease the dose of prednisone. The second case is a 29-year-old Chinese female who presented with a history of membranous nephropathy, epithelioid hemangiomas of the fingers, diffuse lymphadenopathy, and peripheral eosinophilia. She was initially started on tacrolimus and prednisone with inadequate response. MMF was later added to the treatment regime, which resulted in improvement in the lymphadenopathy, and resolution of her proteinuria and peripheral eosinophilia. These two cases add to existing literature that MMF is an effective and well tolerated therapy in this very rare condition.