Successful Immunomodulatory Therapy in Castleman Disease with Paraneoplastic Pemphigus Vulgaris

Castleman disease is a rare lymphoproliferative disorder. The clinical signs and symptoms of the disease are primarily mediated by cytokines, especially interleukin-6. We presented the case of a young female. In May 2004, a 30-year-old otherwise healthy looking woman presented with oral ulcerations resistant to topical and systemic antibiotic and antimycotic treatment. Bullous mucosal lichen or pemphigus vulgaris were suspected. Histological examination and direct and indirect immunofluorescence confirmed the diagnosis of pemphigus. Search for neoplasm revealed a retroperitoneal Castleman tumour sized 15 x 6 x 5 cm in the abdominal MRI. The tumour was a bleeder, so the removal was partial. Histological examination showed hyalin hypervascular Castleman disease. Considering her young, fertile age and the multicentric Castleman disease,