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Updated: May 24, 2026
12:23
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Interstitial pneumonia associated with bullous pemphigoid
Daisuke Yoshioka1, Hiroshi Ishii1, Tomohisa Uchida2
1Department of Internal Medicine II, Oita University Faculty of Medicine, Oita.
Chest
|March 8, 2012
Summary
Bullous pemphigoid, an autoimmune blistering disease, can affect the lungs. This case report details the first immunologically confirmed instance of interstitial pneumonia associated with bullous pemphigoid.
Area of Science:
- Immunodermatology
- Pulmonology
- Autoimmune Diseases
Background:
- Bullous pemphigoid is the most common autoimmune blistering disease.
- It involves an autoimmune attack on hemidesmosome components at the dermal-epidermal junction.
- Immunofluorescence typically shows linear IgG and C3 deposition in skin basement membranes.
Observation:
- A 73-year-old woman presented with interstitial lung disease and skin bullae.
- Symptoms included persistent dry cough and exertional dyspnea.
- Skin lesions were observed on her trunk and extremities.
Findings:
- Chest CT, BAL fluid analysis, and lung biopsy suggested a nonspecific interstitial pneumonia pattern.
- Direct immunofluorescence revealed linear IgG and C3 deposition in both lung and skin basement membranes.
- This confirmed an immune-mediated process affecting both organs.
Implications:
- Lung involvement in bullous pemphigoid is exceptionally rare.
- This is the first reported case with immunological confirmation of interstitial pneumonia.
- Highlights the potential for systemic autoimmune manifestations in bullous pemphigoid.
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