Paraneoplastic pemphigus, an atypical presentation of Castleman's disease

Abstract

Castleman's disease is a non-clonal lymphoproliferative disorder with a wide spectrum of clinical manifestations. We present the clinical case of a male patient who presented asthenia, adynamia, hyporexia, weight loss, oral and genital ulcers, ocular pruritus and conjunctival hyperemia. Physical examination revealed conjunctival hyperemia, oral and genital ulcers. A contrast computed tomography of the thorax and abdomen revealed multiple mediastinal and retroperitoneal lymphadenopathies as well as a solid mass of 94x51 mm retroperitoneally located, which is subsequently biopsied, showing Castleman's disease hyaline vascular variant. A biopsy of the scrotal lesions was also indicated, which had an histopathological analysis consistent with pemphigus. Furthermore, the direct immunological fluorescence was positive in the intercellular spaces of the epidermis as well as the immunoprecipitation with anti-desmoglein, anti-desmoplakin, anti-envoplakin and bullous pemphigoid antigen. As a consequence of the above described, the existence of multicentric Castleman's disease associated with paraneoplastic pemphigus was defined.