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Paraneoplastic Pemphigus Associated With Bronchiolitis Obliterans*

Michiyo Takahashi, MD, PhD; Yoshinori Shimatsu, MD; Takashi Kazama, MD, PhD; Kakuhei Kimura, MD; Tomio Otsuka, MD; Takashi Hashimoto, MD, PhD
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*From the Department of Dermatology (Drs. Takahashi and Kazama), Niigata University School of Medicine, Niigata, Japan; the Department of Dermatology (Dr. Takahashi), the Department of Internal Medicine (Drs. Shimatsu and Otsuka), and the Department of Pathology (Dr. Kimura), Shibata Prefectural Hospital, Shibata, Niigata, Japan; and the Department of Dermatology (Dr. Hashimoto), Kurume University School of Medicine, Kurume, Fukuoka, Japan.

Correspondence to: Michiyo Takahashi, MD, PhD, Department of Dermatology, Shibata Prefectural Hospital, 4–5-48 Ootemachi, Shibata, Niigata 957-0052, Japan; e-mail: michiyo@sbt.lamen.or.jp



Chest. 2000;117(2):603-607. doi:10.1378/chest.117.2.603
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Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. Autoantibodies of PNP bind to the plakin family of cytoplasmic proteins and desmogleins of cell-surface target antigens. We describe a 36-year-old female patient with PNP who had non-Hodgkin’s lymphoma, and who developed bronchiolitis obliterans and died of respiratory failure. Autopsy findings confirmed luminal narrowing of bronchioles by scarring, which is a histopathologic features of bronchiolitis obliterans. After the onset of respiratory failure, the reaction of autoantibodies against the plakins detected by immunoprecipitation at the onset of PNP disappeared with negative immunofluorescence within the bronchial epithelium. It is thought that autoantibodies against some of these antigens play a role in causing acute inflammation of the respiratory epithelium. In treating PNP, the possibility of the patient developing the lethal complication bronchiolitis obliterans should be kept in mind. Furthermore, prevention of the initial autoantibody-mediated injury to the respiratory epithelium should be an important treatment goal.

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