Paraneoplastic pemphigus associated with metastatic lymphoepithelioma-like carcinoma originating from the thyroid gland

Hong, Won Jin; Lee, Sangeun; Chang, Sung Eun; Hashimoto, Takashi; Kim, Soo Chan

doi:10.1111/bjd.13334

Cited by 7 publications

(3 citation statements)

References 11 publications

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“…The pathogenesis of oral lesions in PNP remains unclear, but the presence of autoantibodies against Dsg3 and/or cell-mediated immune responses may play a role in damaging mucosal epithelia9. In general, removal of the underlying neoplasm, especially when the tumors are benign, may lead to clinical improvement and prolonged survival in patients with PNP.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2017

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Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis. Immunoblotting and enzyme-linked immunosorbent assays revealed that the patient had multiple autoantibodies against desmoglein 1, desmocollin 1, 2, 3, laminin gamma-1, envoplakin, and periplakin. The skin lesions completely healed following thymectomy and systemic corticosteroid therapy, but the oral ulcerations persisted through a follow-up period of over 2 years.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2017

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“…Given the fact that Castleman disease has an extremely low incidence in the general population, cases of PNP with Castleman disease are highly frequent. A minor fraction of neoplasms associated with PNP represents non-hematologic neoplasms, including neoplasms originating from the thymus (e.g., thymoma), sarcoma, malignant melanoma, and various epithelial-origin carcinomas (e.g., adenocarcinoma and squamous cell carcinoma) (10, 14, 20, 24, 25). Some cases of PNP were diagnosed before an underlying malignancy was detected (26–28).…”

Section: Disease Manifestationsmentioning

confidence: 99%

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

Kim

2019

Front. Immunol.

172

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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.

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“…Among the non-hematological neoplasms, sarcomas present approximately 8.6% of the cases, such as leiomyosarcoma, malignant nerve sheath tumor, poorly differentiated sarcoma, reticular cell sarcoma, dendritic cell sarcoma, liposarcoma, and inflammatory myofibroblastoma [15][16][17]. Other less common diseases described in the literature that provide PNP are malignant thymoma, squamous cell carcinoma of the esophagus, colon carcinoma, CD8+ T-cell lymphoma, retroperitoneal Kaposi's sarcoma, and lymphoepithelioma-like carcinoma [18][19][20][21][22][23]. Although the PNP is triggered by several neoplasias, the manifestations of this disease may precede the hematological disorders and other malignancies, thus requiring the frequent and continuous follow-up of these patients [15].…”

Section: Association With Malignancy and Genetic Backgroundmentioning

confidence: 99%

Paraneoplastic Pemphigus Is a Life-Threatening Disease

Konichi-Dias¹

2019

Current Trends in Cancer Management

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Paraneoplastic pemphigus is a multiorganic autoimmune disease, usually triggered by neoplasias, mainly of lymphoproliferative origin such as chronic lymphocytic leukemia, multiple myeloma, non-Hodgkin's lymphoma, Castleman disease, and thymoma. This disorder is characterized by the presence of autoantibodies that react against proteins, such as desmoplakins, desmocollins, and others existing in cell junctions. The prognosis is reserved, and the mortality rate of the disease is very high, thus proving to be an additional challenge in the therapeutic management of onco-hematological diseases. The objective of this chapter is to solve the main clinical aspects of paraneoplastic pemphigus in lymphoproliferative hematological diseases, anatomopathological and immunofluorescence characteristics, as well as associations with the main differential diagnoses and therapeutic management. We will also describe the main differential diagnoses of paraneoplastic pemphigus, such as various types of pemphigus including induced drug, bullous pemphigoid, drug eruption, lichen planus, graft versus host disease, erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. In addition, the prognosis and quality of life will be mentioned.

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Paraneoplastic pemphigus associated with metastatic lymphoepithelioma-like carcinoma originating from the thyroid gland

Cited by 7 publications

References 11 publications

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

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

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

Paraneoplastic Pemphigus Is a Life-Threatening Disease

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