ABSTRACT
Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis, which is caused by potential neoplasm, especially the Castleman's disease. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices. Furthermore, it is reported that the mortality rate for this disease is very high. Bronchiolitis obliterans is the most common complication and the most important cause of death. There was a female patient presenting recalcitrant mucocutaneous erosions, ulcers and scattered erythemas in the body. The patient was diagnosed and treated for pemphigus vulgaris with little success in Xiangya Hospital, Central South University in January 2015. Further investigations confirmed the diagnosis of paraneoplastic pemphigus with retroperitoneal tumor. Subsequently, the patient was treated with tumor resection in combination with intravenous immunoglobulin and corticosteroids. The pathology revealed that it was the Castleman's disease. Her mucocutaneous performance recovered obviously and the bronchiolitis obliteran did not appear in the follow-up. Castleman's disease associated with paraneoplastic pemphigus should be considered when mucosal and skin lesions showing no improvement under corticosteroids. Early and complete removal of the tumor together with immunotherapy could be beneficial to the patient's prognosis.
Subject(s)
Female , Humans , Adrenal Cortex Hormones , Therapeutic Uses , Castleman Disease , Therapeutics , Immunoglobulins, Intravenous , Therapeutic Uses , Paraneoplastic Syndromes , Therapeutics , Pemphigus , Therapeutics , Retroperitoneal Space , PathologyABSTRACT
Poorly differentiated thyroid carcinoma (PDTC) is a special type of thyroid carcinoma, the morphology and biological behavior of which are between well-differentiated and undifferentiated (anaplastic) carcinomas. Currently, the diagnosis of PDTC mainly relies on the pathological standards. Although "Turin standards" is commonly used, there is no generally accepted diagnostic criteria. Surgery is still the main treatment for PDTC, but the adjuvant therapies are in dispute. Age, tumor node metastasis (TNM) stage and integrity of surgical of PDTC are major factors that affect the prognosis. The identification of eosinophilic phenotype (hurthle cells) of PDTC is important. Some common immunohistochemical and molecular biomarkers, such as the insulin-like growth factor II mRNA-binding protein 3 (IMP3), E-cadherin and proliferating protein Ki67, may be helpful for distinguishing PDTC from other thyroid carcinoma. With the progress in studies regarding molecular markers for PDTC and the clinical characters of PDTC patients with large samples, the diagnosis for PDTC will greatly improved and the pathogenesis for PDTC will be elucidated.