Antibody-negative paraneoplastic autoimmune multiorgan syndrome (PAMS) in a patient with follicular lymphoma accompanied by an excess of peripheral blood CD8+ lymphocytes
- 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.
Author: | Thilo GambichlerORCiDGND, Yi-Pei LeeGND, Ilske OschliesGND, Christina ScheelORCiDGND, Wolfram KlapperGND, Nico NowackGND, Martin DörlerGND, Markus StückerGND, Nasreddin AbolmaaliGND, Laura SusokGND |
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URN: | urn:nbn:de:hbz:294-101770 |
DOI: | https://doi.org/10.3390/curroncol29040194 |
Parent Title (English): | Current oncology |
Publisher: | MDPI |
Place of publication: | Basel |
Document Type: | Article |
Language: | English |
Date of Publication (online): | 2023/09/19 |
Date of first Publication: | 2022/03/28 |
Publishing Institution: | Ruhr-Universität Bochum, Universitätsbibliothek |
Tag: | Open Access Fonds autoantibodies; autoimmune blistering diseases; cancer; cytotoxic lymphocytes; paraneoplastic pemphigus |
Volume: | 29 |
Issue: | 4 |
First Page: | 2395 |
Last Page: | 2405 |
Note: | Article Processing Charge funded by the Deutsche Forschungsgemeinschaft (DFG) and the Open Access Publication Fund of Ruhr-Universität Bochum. |
Institutes/Facilities: | Katholisches Klinikum Bochum, Dermatologie, Venerologie und Allergologie |
Dewey Decimal Classification: | Technik, Medizin, angewandte Wissenschaften / Medizin, Gesundheit |
open_access (DINI-Set): | open_access |
Licence (English): | Creative Commons - CC BY 4.0 - Attribution 4.0 International |