Bronchiolitis Obliterans in a Young Female Patient with Paraneoplastic Pemphigus Associated with Unicentric Castleman’s Disease

Background: Castleman’s disease (CD), also known as angiofollicular lymph node hyperplasia, describes a rare group of diseases manifesting with enlarged lymph nodes and various inflammatory symptoms. The association between Castleman’s disease, paraneoplastic pemphigus and bronchiolitis obliterans has been described in the literature and is depicted thoroughly in this case. Case Presentation: We present a case of severe bronchiolitis obliterans developing in a 17-year-old female with paraneoplastic pemphigus and unicentric Castleman’s disease. Conclusion: Surgical resection of unicentric Castleman’s disease remains the treatment of choice due to its efficacy in preventing the recurrence of associated morbidity caused by bronchiolitis obliterans and paraneoplastic pemphigus.

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Publication Details

Journal
Lymphatics
Published
2025-09-24
DOI
https://doi.org/10.3390/lymphatics3040030
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
Field-Weighted Citation Impact
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article

Bronchiolitis Obliterans in a Young Female Patient with Paraneoplastic Pemphigus Associated with Unicentric Castleman’s Disease

Dirk Theegarten, Anna Daniels, Stefanie Werther, Enno Schmidt et al.
Lymphatics
Autoimmune Bullous Skin Diseases
article

Bronchiolitis Obliterans in a Young Female Patient with Paraneoplastic Pemphigus Associated with Unicentric Castleman’s Disease

Dirk Theegarten, Anna Daniels, Stefanie Werther, Enno Schmidt, Silke Hofmann, V. Besa, Walid Kteiche, Stefanie Bertram
article en

Abstract

Background: Castleman’s disease (CD), also known as angiofollicular lymph node hyperplasia, describes a rare group of diseases manifesting with enlarged lymph nodes and various inflammatory symptoms. The association between Castleman’s disease, paraneoplastic pemphigus and bronchiolitis obliterans has been described in the literature and is depicted thoroughly in this case. Case Presentation: We present a case of severe bronchiolitis obliterans developing in a 17-year-old female with paraneoplastic pemphigus and unicentric Castleman’s disease. Conclusion: Surgical resection of unicentric Castleman’s disease remains the treatment of choice due to its efficacy in preventing the recurrence of associated morbidity caused by bronchiolitis obliterans and paraneoplastic pemphigus.

LymphaticsVol. 3(4)
Witten/Herdecke University (DE), Ruhrlandklinik (DE), Essen University Hospital (DE), Helios Universitätsklinikum Wuppertal (DE), University of Lübeck (DE)
Openalex Percentile: Top 63%
Autoimmune Bullous Skin Diseases
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