Pemphigus vulgaris-associated interstitial lung disease

Dermatol Ther. 2016 Jul;29(4):228-32. doi: 10.1111/dth.12342. Epub 2016 Mar 11.


Autoimmune bullous diseases (AIBDs)-associated interstitial lung disease (ILD) is extremely rare. Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease caused by circulating autoantibodies against desmoglein. To date, PV-associated ILD has rarely been reported in English literature. We report a rare association of PV and ILD. A 53-year-old Chinese female with PV for 8 months developed ILD after a relapse of PV for 2 months due to discontinuation of oral prednisone by herself. She was successfully treated by systemic methylprednisolone. Taken previously reported bullous pemphigoid-associated ILD and linear IgA/IgG bullous dermatosis-associated ILD together, in general, AIBDs-associated ILD occurs when AIBDs relapse or are not controlled, responds well to systemic corticosteroids, and has a relatively better prognosis when compared with rheumatoid arthritis- or dermatomyositis-associated ILD.

Keywords: chemokine; desmoglein; ferritin; interleukin 18; interstitial lung disease; pemphigus vulgaris.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Humans
  • Lung Diseases, Interstitial / etiology*
  • Lymphopenia / etiology
  • Middle Aged
  • Pemphigus / complications*
  • Pemphigus / pathology
  • Prednisolone / therapeutic use


  • Prednisolone