1例Castleman病合并副肿瘤性天疱疮患者的诊治

A case of retroperitoneal Castleman's disease with paraneoplastic pemphigus

副肿瘤性天疱疮是一种由潜在肿瘤引起的自身免疫性大疱性皮肤病,以Castleman病合并副肿瘤性天疱疮尤为常见,临床上很容易误诊。2015年1月中南大学湘雅医院收治了1例表现为顽固性皮肤黏膜糜烂的患者,起初以"寻常型天疱疮"治疗,效果不佳,进一步检查发现潜在的腹膜后肿瘤,考虑可能为Castleman病合并副肿瘤性天疱疮。予以手术切除联合免疫球蛋白和糖皮质激素治疗,术后病检提示为Castleman病,后来患者皮肤黏膜病变明显好转。当皮肤黏膜病变经药物治疗无明显改善时,应考虑Castleman病合并副肿瘤性天疱疮的可能,尽早完整切除肿瘤联合免疫治疗可明显改善副肿瘤性天疱疮患者的预后。

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.