Improved outcomes after complete resection of underlying tumors for patients with paraneoplastic pemphigus: a single-center experience of 22 cases

Abstract

Early detection, prompt treatment, and complete resection of PNP can effectively decrease the mortality and speed up the recovery.

Robotic trans-subxiphoid extended thymectomy in a patient with thymoma-associated pemphigus

“…In previous reports, the majority of thymoma in patients with TAP were non-invasive and complete thymectomy was demonstrated as effective for this disease. A TAP patient reported by Zhang et al (5) was successfully treated with complete removal of the thymoma after presenting with preoperative respiratory symptoms. Winkler et al (6) published a case report about a patient with a rare triad of thymoma, myasthenia, and TAP.…”

Paraneoplastic Autoimmune Multiorgan Syndrome: Paraneoplastic Pemphigus Revisited

“…Complete resection of 20 cases of neoplasia associated with PAMS led to sustained mucocutaneous improvement in 15 patients; however, respiratory symptoms and compromised pulmonary function persisted in the majority of these cases [ 53 ]. Pulmonary involvement is often progressive and irreversible despite tumor resection and treatment of mucocutaneous disease.…”

“…In a review of 163 cases, non-Hodgkin's lymphoma (38.6 %), chronic lymphocytic leukemia (18.4 %), Castleman's disease (18.4 %), and thymoma (5.5 %) are the most commonly reported concurrent neoplasms [ 7 ]. Castleman's disease appears to be reported in much higher frequency in children [ 2 , 8 , 9 ] and in Chinese PAMS (77 %) [ 10 ]. Of several cases reported to occur in association with thymoma and Castleman's disease, further pathologic examination revealed concurrent follicular dendritic cell sarcoma (FDCS) [ 11 , 12 ].…”

Paraneoplastic pemphigus in Russian patients: a single center case series

“…Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous disease with mortality rates up to 90% . Diagnostic criteria for PNP include progressive stomatitis, polymorphic lesions, an underlying neoplasm, specific histological changes, deposition of IgG and complement in the epidermal intercellular spaces, and presence of specific autoantibodies . As of yet, 250–450 cases have been reported in the literature .…”