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[Paraneoplastic pemphigus with tracheobronchial involvement]

M Segard-Drouard1, I Lefebvre, B Catteau

  • 1Clinique Dermatologique, Hôpital Claude Huriez, CHRU, Lille.

Annales De Dermatologie Et De Venereologie
|January 1, 1997
PubMed
Summary

Paraneoplastic pemphigus, a rare autoimmune blistering disease, can affect the airways. This case highlights the diagnostic challenges and poor prognosis associated with tracheobronchial involvement in chronic lymphoid leukemia patients.

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Area of Science:

  • Dermatology
  • Immunology
  • Oncology

Background:

  • Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease with distinct clinical, histological, and immunological features.
  • It is frequently associated with lymphoproliferative disorders, presenting a diagnostic challenge due to its varied manifestations.

Observation:

  • A patient with chronic lymphoid leukemia (CLL) presented with mucocutaneous lesions, including erosions and bullae, initially mimicking Stevens-Johnson syndrome.
  • Histopathology revealed intraepidermal blistering and suprabasal acantholysis, with direct immunofluorescence showing intercellular IgG and C3 deposits.
  • Immunotransfer confirmed PNP by detecting antibodies against specific antigens (250, 230, 210, 190 kD).

Findings:

  • The patient subsequently developed respiratory symptoms, with bronchial endoscopy revealing ulceronecrotic plaques and tracheal biopsy showing acantholytic cells.

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  • Autopsy confirmed epithelial cleavage and acantholysis involving the trachea and bronchi, indicating extensive tracheobronchial involvement.
  • Corticosteroid therapy improved skin lesions but was insufficient to prevent rapid deterioration and death from pneumonia.

    • Implications:

    • This case underscores the difficulty in early PNP diagnosis, especially when oral and genital lesions mimic other conditions.
    • Tracheobronchial involvement in PNP, even without clear immunological markers, significantly worsens the prognosis and requires thorough investigation in patients with bronchopulmonary symptoms.
    • Early recognition and management of airway involvement are critical for improving outcomes in paraneoplastic pemphigus.