Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: A rare case of Castleman's disease with respiratory failure requiring lung transplantation

J Pediatr Surg. 2001 Dec;36(12):E22. doi: 10.1053/jpsu.2001.28877.


Castleman's disease is an infrequent and usually benign lymphoproliferative disorder. Resection of the tumor usually is curative. The immunostimulatory nature of the tumor can, in rare instances, result in paraneoplastic manifestations. The authors present a case of a 14 year old with mucocutaneous ulcerations and progressive dyspnea that was found to have a large mediastinal mass and circulating autoantibodies that were responsible for his paraneoplastic pemphigus and bronchiolitis obliterans. In spite of aggressive immunotherapy to control the autoimmune mucocutaneous lesions, the pulmonary fibrosis was irreversible and progressed to pulmonary failure necessitating lung transplantation. J Pediatr Surg 36:E22.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Bronchiolitis Obliterans / diagnosis
  • Bronchiolitis Obliterans / pathology
  • Bronchiolitis Obliterans / surgery*
  • Castleman Disease / diagnosis
  • Castleman Disease / pathology
  • Castleman Disease / surgery*
  • Humans
  • Lung Transplantation*
  • Male
  • Mediastinal Neoplasms / diagnosis
  • Mediastinal Neoplasms / pathology
  • Mediastinal Neoplasms / surgery*
  • Paraneoplastic Syndromes / diagnosis
  • Paraneoplastic Syndromes / pathology
  • Paraneoplastic Syndromes / surgery*
  • Pemphigus / diagnosis
  • Pemphigus / pathology
  • Pemphigus / surgery*