Paraneoplastic Pemphigus Occurs Most Commonly in Indolent B Cell Lymphoma

Morikawa, Keiko; Tsuji, Takeshi; Yamasaki, Hiroshi; Egashira, Sho; Kaguchi, Atsushi; Kido, Masato; Tsuda, Hiroyuki

doi:10.1159/000357109

Cited by 9 publications

(3 citation statements)

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“…The initial symptoms of gastrointestinal follicular lymphoma manifestations include obstipation, abdominal pain, and intestinal bleeding. However, follicular lymphoma may also remain asymptomatic over a longer period [8][9][10][11]. With respect to the present case, we would like to argue that asymptomatic follicular lymphoma was the initial and persistent immunological trigger of the mucocutaneous lesions that arose, for the first time, in July 2020.…”

Section: Discussionmentioning

confidence: 88%

“…Here, we describe a patient who was initially diagnosed with vaccine-induced chronically recurrent SJS, accompanied by a chronic excess of peripheral CD8+ lymphocytes. One and a half years later, following the detection of follicular lymphoma, the patient was finally diagnosed with antibody-negative PAMS, with a fatal outcome three weeks later [7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

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Antibody-Negative Paraneoplastic Autoimmune Multiorgan Syndrome (PAMS) in a Patient with Follicular Lymphoma Accompanied by an Excess of Peripheral Blood CD8+ Lymphocytes

et al. 2022

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Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a life-threatening autoimmune disease associated with malignancies. Here, we present a patient initially misdiagnosed with “chronic” Stevens–Johnson syndrome. Over a year later, the patient was diagnosed with stage IV follicular lymphoma and treated with an anti-CD20 antibody. At this time, his skin condition had significantly worsened, with erythroderma and massive mucosal involvement, including in the mouth, nose, eyes, and genital region. Histopathology revealed lichenoid infiltrates with interface dermatitis, dyskeratoses, necrotic keratinocytes, and a dense CD8+ infiltrate with strong epidermotropism. Direct and indirect immunofluorescence tests for autoantibodies were negative. Remarkably, we retrospectively discovered a chronic increase in peripheral CD8+ lymphocytes, persisting for over a year. Consequently, the patient was diagnosed with antibody-negative PAMS. Three weeks later, he succumbed to respiratory failure. This dramatic case highlights the challenges in diagnosing PAMS, particularly in cases where immunofluorescence assays are negative. Importantly, we observed, for the first time, a chronic excess of CD8+ peripheral blood lymphocytes, associated with PAMS, consistent with the systemic, autoreactive T-cell-driven processes that characterize this condition.

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Section: Discussionmentioning

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Antibody-Negative Paraneoplastic Autoimmune Multiorgan Syndrome (PAMS) in a Patient with Follicular Lymphoma Accompanied by an Excess of Peripheral Blood CD8+ Lymphocytes

et al. 2022

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“…Eighty-nine percent of the NHL associated with PNP are of B cell type. NHL subtypes includes CLL (43 %), FL (27 %), T-cell lymphoma (11 %), indolent B-cell lymphoma (7 %) and B-cell lymphoma unspecified (7 %) and Diffuse large B-cell lymphoma (5 %) indicating that indolent NHL is most commonly associated with PNP [8]. DIF shows IgG and C3 deposition in inter-cellular areas and may also show linear/granular deposition along basement membrane [6].…”

mentioning

confidence: 99%