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Thymoma with pemphigus foliaceus.

K Takeshita1, M Amano, T Shimizu

  • 1Department of Medicine, Keio University School of Medicine, Tokyo.

Internal Medicine (Tokyo, Japan)
|September 2, 2000
PubMed
Summary

A thymoma-related skin condition, pemphigus foliaceus, improved significantly after surgical removal of a thymoma. This suggests thymectomy may benefit patients with this rare autoimmune blistering disease.

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Area of Science:

  • Dermatology and Oncology
  • Autoimmune blistering diseases
  • Thymoma and associated paraneoplastic syndromes

Background:

  • Pemphigus foliaceus is a rare autoimmune blistering disease.
  • Anterior mediastinal masses can rarely be associated with paraneoplastic syndromes.
  • The co-occurrence of thymoma and pemphigus foliaceus is exceptionally uncommon.

Observation:

  • A 75-year-old woman presented with an anterior mediastinal mass and a diagnosis of pemphigus foliaceus.
  • Skin lesions included crusts and erosions on the chest, back, and scalp.
  • Serum analysis confirmed positivity for anti-desmoglein 1 antibodies.

Findings:

  • Histopathological examination of skin biopsies confirmed pemphigus foliaceus.
  • Surgical resection of the anterior mediastinal mass revealed a mixed lymphoepithelial thymoma.

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  • One year post-thymectomy, the patient experienced significant improvement in skin eruption and alopecia, with decreased anti-desmoglein 1 antibody titers.

    • Implications:

    • This case suggests a potential paraneoplastic link between thymoma and pemphigus foliaceus.
    • Thymectomy may be a beneficial therapeutic option for patients with thymoma-associated pemphigus foliaceus.
    • Further research is warranted to elucidate the immunopathogenesis of this rare association.