Paraneoplastic pemphigus involved oral mucosa only: a case report and literature review

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. A chest computed tomography (CT) scan revealed Castleman disease. Following surgical removal of the tumor, the patient's oral mucosa healed completely, with no recurrence observed in a 4-year follow-up period. These findings emphasize the importance of considering PNP as a potential diagnosis in patients with refractory oral erosions displaying lichen planus-like and pemphigus-like features, particularly when unresponsive to corticosteroid therapy. A thoracic-abdominal-pelvic CT scan is recommended to facilitate accurate diagnosis and appropriate treatment.

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Publication Details

Journal
Frontiers in Medicine
Published
2025-01-27
DOI
https://doi.org/10.3389/fmed.2025.1498800
Citations
1
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
Field-Weighted Citation Impact
1.47

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article

Paraneoplastic pemphigus involved oral mucosa only: a case report and literature review

Mei Yang, Tingting Zhu, Yang Liu, Caixia Wang et al.
1 citations
Frontiers in Medicine
Autoimmune Bullous Skin Diseases
1.47
article

Paraneoplastic pemphigus involved oral mucosa only: a case report and literature review

Mei Yang, Tingting Zhu, Yang Liu, Caixia Wang, Wanchun Wang, Xixi Yu
article en
1 citations

Abstract

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. A chest computed tomography (CT) scan revealed Castleman disease. Following surgical removal of the tumor, the patient's oral mucosa healed completely, with no recurrence observed in a 4-year follow-up period. These findings emphasize the importance of considering PNP as a potential diagnosis in patients with refractory oral erosions displaying lichen planus-like and pemphigus-like features, particularly when unresponsive to corticosteroid therapy. A thoracic-abdominal-pelvic CT scan is recommended to facilitate accurate diagnosis and appropriate treatment.

Frontiers in MedicineVol. 12
Qingdao University (CN), Peking University (CN)
Qingdao Key Health Discipline Development Fund, Qingdao University
Openalex Percentile: Top 23%
Autoimmune Bullous Skin Diseases
1.47
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