Bai Yi-Xiu, Chu Jin-Gang, Xiao Ting, Chen Hong-Duo
Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China.
Department of Radiology, No. 1 Hospital of China Medical University, Shenyang, China.
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.
