Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy

Lim, Jung Min; Lee, Sangeun; Seo, Jeong Taeg; Kim, Do Young; Hashimoto, Takashi; Kim, Soo Chan

doi:10.5021/ad.2017.29.2.219

Cited by 19 publications

(24 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These lung functions suggested small airway lesions and possible interstitial damage. The most common characteristic of CD accompanied by PNP is stomatitis [24,25], which is usually the rst symptom of the disease and persists throughout the course of the disease. Stomatitis is manifested as mucosal erosion and ulceration, which is the most common in HV-UCD.…”

Section: Discussionmentioning

confidence: 99%

WITHDRAWN: Lung Involvement Multicentric Castleman of Hyaline-vascular Variant: 3 cases

Cen¹,

Chen

Zeng

et al. 2021

Preprint

View full text Add to dashboard Cite

BackgroundCastleman disease is clinically divided into unicentric Castleman disease (UCD), with hyaline-vascular Variant (HV) as major pathological pattern, and multicentric Castleman disease (MCD), with plasma cell type (PC) most commonly. Lung involvement is a serious complication of CD and usually leads to death.MethodWe retrospectively analyzed the medical records of 3 patients admitted to The First Affiliated Hospital of Guangxi Medical University during January 1, 2007 to September 30, 2020.Result2 male and 1 female with age ranging from 16 years old to 63 years old were admitted. The involved areas varied a lot. Respiratory symptoms were seen in 3 cases, along with fever, weight loss and splenomegaly. Dry and wet rales were found in all patients. All patients had hypoxemia and obstructive ventilation dysfunction. Computed tomography (CT) mainly showed bronchiectasis and mediastinal lymph node enlargement. All 3 cases were complicated with PNP. 1 case failed chemotherapy after local mass excision, 1 case remitted after chemotherapy but the lung lesion was irreversible, and 1 case was untreated and soon die of respiratory failure.ConclusionCases of HV-MCD with pulmonary affected were characterized by small airway lesions and poor prognosis. Respiratory symptoms along with systemic symptoms were common.

show abstract

Section: Discussionmentioning

confidence: 99%

WITHDRAWN: Lung Involvement Multicentric Castleman of Hyaline-vascular Variant: 3 cases

Cen¹,

Chen

Zeng

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Paraneoplastic pemphigus is a group of life-threatening autoimmune blistering skin disorders that can occur in cancer patients. Thymectomy can decrease antibody titer and improve clinical outcomes in thymoma-related pemphigus (32,33). In this study, one pemphigus patient who underwent surgery after six months of steroid treatment did not experience a disease flare.…”

Section: Discussionmentioning

confidence: 67%

Thymic epithelial tumor complicated by immunological abnormalities: results from a single-center retrospective study in China

et al. 2019

View full text Add to dashboard Cite

Background: To describe the clinical manifestations, immunological features, treatments, and outcomes of patients with thymic epithelial tumor (TET) complicated by immunological abnormalities, and to improve knowledge on immunological abnormalities in this rare disease. Methods: Patients with pathologically confirmed TET at Beijing Chaoyang Hospital between January 2013 and May 2018 were included in this study, and clinical data were analyzed retrospectively. Immunological abnormalities were classified into two groups as follows: Good syndrome (GS) and autoimmune disease (AD). Results: Fifty-nine TET patients were enrolled; twenty-two patients (37.3%) had immune dysfunction. There were no gender, age, or histological type differences between groups with or without immunological abnormalities. Six patients had GS, of whom four patients were diagnosed after thymectomy. Recurrent respiratory infections, particularly opportunistic infections, were the most common manifestation. Three GS patients developed a second cancer (50%; P=0.011). Anti-infective therapy and immunoglobulin supplements effectively treated GS. Seventeen patients developed ADs, including myasthenia gravis (MG) (n=13), Hashimoto's thyroiditis (n=4), Sjogren's syndrome (n=1), rheumatoid arthritis (n=1), pemphigus (n=1), and Evans syndrome (n=1). One patient developed both MG and GS and 4 patients presented with two ADs. Three AD cases occurred after thymectomy. Pemphigus and 80% (8/10) of MG cases were resolved following thymectomy. Conclusions: There is a strong association between immunological abnormalities and TET, which may present at any time point during the disease, even after thymectomy. In addition to infection, GS patients are more likely to develop a second cancer. Thymectomy may produce favorable outcomes for MG in this study, while surgery does not improve immunodeficiency in GS patients.

show abstract

“…The most characteristic feature of PNP is stomatitis, which usually is the first presenting sign and persists over the course of the disease (2, 12). Stomatitis presents as erosions and ulcerations affecting the oropharynx and extending to the vermilion border of the lips (Figure 1A).…”

Section: Disease Manifestationsmentioning

confidence: 99%

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

Kim

2019

Front. Immunol.

Self Cite

172

View full text Add to dashboard Cite

Paraneoplastic pemphigus (PNP) is a rare but life-threatening mucocutaneous disease mediated by paraneoplastic autoimmunity. Various neoplasms are associated with PNP. Intractable stomatitis and polymorphous cutaneous eruptions, including blisters and lichenoid dermatitis, are characteristic clinical features caused by humoral and cell-mediated autoimmune reactions. Autoreactive T cells and IgG autoantibodies against heterogeneous antigens, including plakin family proteins and desmosomal cadherins, contribute to the pathogenesis of PNP. Several mechanisms of autoimmunity may be at play in this disease on the type of neoplasm present. Diagnosis can be made based on clinical and histopathological features, the presence of anti-plakin autoantibodies, and underlying neoplasms. Immunosuppressive agents and biologics including rituximab have been used for the treatment of PNP; however, the prognosis is poor due to underlying malignancies, severe infections during immunosuppressive treatment, and bronchiolitis obliterans mediated by autoimmunity. In this review, we overview the characteristics of PNP and focus on the immunopathology and the potential pathomechanisms of this disease.

show abstract

Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy

Cited by 19 publications

References 9 publications

WITHDRAWN: Lung Involvement Multicentric Castleman of Hyaline-vascular Variant: 3 cases

WITHDRAWN: Lung Involvement Multicentric Castleman of Hyaline-vascular Variant: 3 cases

Thymic epithelial tumor complicated by immunological abnormalities: results from a single-center retrospective study in China

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

Contact Info

Product

Resources

About