Summarize the clinical characteristics, treatment methods, and prognosis of paraneoplastic pemphigus with oral lesions, and identify commonalities and features to provide better references for the clinical diagnosis and treatment of this disease. A retrospective analysis was conducted on the case data and clinical photos of 9 patients with paraneoplastic pemphigus accompanied by oral lesions treated in the Internal Medicine Ward and Department of Stomatology of Beijing Hospital from September 2017 to December 2023, and the clinical characteristics were summarized and analyzed. All 9 patients had oral mucosal and skin lesions simultaneously, in whom visceral tumors were found upon systemic examinations, including 4 cases of Castleman's disease, 4 cases of thymoma, and 1 case of lung cancer. All patients had received glucocorticoid therapy before a clear diagnosis, but the effect was not significant. After removing the primary tumor, corticosteroid intravenous or oral therapies were usually continued, supplemented with immunoglobulin and immunosuppressants for treatment, which could achieve significant therapeutic effects. With primary tumor resection, 6 patients continued to receive corticosteroid intravenous or oral treatment for 6 to 12 months, supplemented with immunoglobulin and immunosuppressants. Oral mucosal erosion improved significantly and skin lesions were relieved. Three patients who were unable to remove their primary tumor continued with the previous glucocorticoid treatment regimen and died withim one year. However, local treatment of their oral cavity during the treatment process still helped alleviate oral mucosal pain, promote erosion healing, and improve nutritional status. Multiple erosions of the oral mucosa and polymorphic skin damages are common clinical manifestations of paraneoplastic pemphigus. Castleman's disease and thymoma are common coexisting tumors. Early diagnosis and timely surgical resection of tumors are crucial for treatment and prognosis. The application of glucocorticoids and local oral treatment after resection is effective.
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http://dx.doi.org/10.3760/cma.j.cn112144-20241008-00375 | DOI Listing |
Arch Iran Med
February 2025
Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Background: Pemphigus consists of a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. Pemphigus includes three major forms: pemphigus vulgaris (PV), pemphigus foliaceus, and paraneoplastic pemphigus. Before the advent of systemic corticosteroids (SCSs), pemphigus was usually a fatal disease.
View Article and Find Full Text PDFZhonghua Kou Qiang Yi Xue Za Zhi
February 2025
Department of Stomatology, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China.
Summarize the clinical characteristics, treatment methods, and prognosis of paraneoplastic pemphigus with oral lesions, and identify commonalities and features to provide better references for the clinical diagnosis and treatment of this disease. A retrospective analysis was conducted on the case data and clinical photos of 9 patients with paraneoplastic pemphigus accompanied by oral lesions treated in the Internal Medicine Ward and Department of Stomatology of Beijing Hospital from September 2017 to December 2023, and the clinical characteristics were summarized and analyzed. All 9 patients had oral mucosal and skin lesions simultaneously, in whom visceral tumors were found upon systemic examinations, including 4 cases of Castleman's disease, 4 cases of thymoma, and 1 case of lung cancer.
View Article and Find Full Text PDFBMJ Case Rep
February 2025
Department of Clinical Hematology and Medical Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
We report a case of a woman in her early 20s who presented with chronic and refractory oro-oesophageal ulcers and significant weight loss. The common causes of refractory ulcers were ruled out. Biopsy from the oesophageal ulcers was suggestive of paraneoplastic pemphigus (PNP).
View Article and Find Full Text PDFFront Med (Lausanne)
January 2025
Department of Oral Medicine, Peking University School and Hospital of Stomatology, Beijing, China.
Paraneoplastic pemphigus (PNP) is a rare and potentially fatal autoimmune disease that primarily affects the mucocutaneous tissues. Here we present the clinical features of a patient with the lichenoid variant of PNP involving the oral mucosa only. The patient exhibited clinical manifestations resembling both lichen planus and pemphigus, yet these lesions remained unresponsive to corticosteroid therapy.
View Article and Find Full Text PDFInt J Surg Case Rep
February 2025
Department of Surgical Oncology, Shaukat Khanum Memorial Cancer Hospital & Research Center, Pakistan; Shaukat Khanum Memorial Cancer Hospital and Research Centre, 7A Block R-3, M.A.Johar Town, Lahore, Pakistan. Electronic address:
Introduction: Castleman disease is a rare lymphoproliferative disorder, subdivided into three types: unicentric Castleman disease, idiopathic multicentric Castleman disease and human herpesvirus-8 (HHV8) associated multicentric Castleman disease. The retroperitoneum comprises only 13 % of the cases.
Case Presentation: We report a case of a 36-year-old female who presented with skin lesions in a dermatology clinic.
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