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Pemphigus vulgaris‐associated interstitial lung disease
Authors:Yi‐Xiu Bai  Jin‐Gang Chu  Ting Xiao  Hong‐Duo Chen
Affiliation:1. Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China;2. Department of Radiology, No. 1 Hospital of China Medical University, Shenyang, China
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.
Keywords:chemokine  desmoglein  ferritin  interleukin 18  interstitial lung disease  pemphigus vulgaris
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