Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[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
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Six Years of Genetic Diagnosis of Severe Early-Onset Obesity in a French Cohort.

Obesity science & practice·2025
Same author

Use of perfusion device for free flap salvage after ischemia in swine.

Annales de chirurgie plastique et esthetique·2024
Same author

Environment and dermatology.

Annales de dermatologie et de venereologie·2022
Same author

A swollen cheek.

Journal of stomatology, oral and maxillofacial surgery·2020
Same author

Single, short in-del, and copy number variations detection in monogenic dyslipidemia using a next-generation sequencing strategy.

Clinical genetics·2018
Same author

KIR3DL2 expression in patients with adult T-cell lymphoma/leukaemia.

The British journal of dermatology·2018

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.

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.

Related Experiment Videos

  • 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.