title: Pemphigus vulgaris-associated interstitial lung disease.
abstract: 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.
authors.affiliation: Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China.
authors.forename: Yi-Xiu
authors.initials: YX
authors.lastname: Bai
authors.affiliation: Department of Radiology, No. 1 Hospital of China Medical University, Shenyang, China.
authors.forename: Jin-Gang
authors.initials: JG
authors.lastname: Chu
authors.affiliation: Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China.
authors.forename: Ting
authors.initials: T
authors.lastname: Xiao
authors.affiliation: Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China.
authors.forename: Hong-Duo
authors.initials: HD
authors.lastname: Chen
mesh_terms: D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology*; D008231:Lymphopenia / Q000209:etiology; D008875:Middle Aged; D010392:Pemphigus / Q000150:complications* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D011239:Prednisolone
keywords: chemokine; desmoglein; ferritin; interleukin 18; interstitial lung disease; pemphigus vulgaris