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Updated: Mar 24, 2026
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Pemphigus vulgaris-associated interstitial lung disease
Yi-Xiu Bai1, Jin-Gang Chu2, Ting Xiao1
1Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China.
Dermatologic Therapy
|March 13, 2016
Summary
Autoimmune bullous diseases-associated interstitial lung disease is rare. Pemphigus vulgaris-associated ILD, a rare condition, responded well to corticosteroids, suggesting a better prognosis than other autoimmune-ILD associations.
Area of Science:
- Pulmonology and Dermatology
- Immunology
Background:
- Autoimmune bullous diseases (AIBDs) are rare causes of interstitial lung disease (ILD).
- Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease characterized by autoantibodies against desmoglein.
- PV-associated ILD is exceptionally rare in English literature.
Observation:
- A 53-year-old Chinese female with a history of PV developed ILD following a PV relapse after discontinuing oral prednisone.
- The patient's ILD was successfully treated with systemic methylprednisolone.
Findings:
- This case highlights a rare association between Pemphigus vulgaris and interstitial lung disease.
- AIBD-associated ILD, including bullous pemphigoid and linear IgA/IgG bullous dermatosis, generally occurs during disease relapse or poor control.
- These conditions typically respond well to systemic corticosteroids.
Implications:
- AIBD-associated ILD may have a better prognosis compared to ILD associated with rheumatoid arthritis or dermatomyositis.
- Early recognition and prompt corticosteroid treatment are crucial for managing AIBD-associated ILD.
- Further research is warranted to understand the pathogenesis and long-term outcomes of AIBD-associated ILD.
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