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Original Article
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Volume 340:1406-1410 May 6, 1999 Number 18
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The Mechanism of Respiratory Failure in Paraneoplastic Pemphigus
Hossein C. Nousari, M.D., Robin Deterding, M.D., Henry Wojtczack, M.D., Sirpa Aho, Ph.D., Jouni Uitto, M.D., Ph.D., Takashi Hashimoto, M.D., and Grant J. Anhalt, M.D.

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Paraneoplastic pemphigus1 is an autoimmune disease that accompanies an overt or occult neoplasm and causes blisters. It is characterized by the presence of IgG autoantibodies that react against desmosomal and hemidesmosomal plakin proteins,2,3,4,5 desmosomal transmembrane proteins (desmogleins),6 and an unidentified 170-kd antigen. Blistering of stratified squamous epithelium results from acantholysis, the loss of cell–cell adhesion, induced by pathogenic antibodies against the desmogleins.6 The most commonly associated neoplasms are, in decreasing order of frequency, non-Hodgkin's lymphoma, chronic lymphocytic leukemia, Castleman's disease, thymoma, retroperitoneal sarcomas, and Waldenström's macroglobulinemia.

Progressive respiratory failure with clinical features of bronchiolitis obliterans is frequently the cause of . . . [Full Text of this Article]

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From the Department of Dermatology, Johns Hopkins University, Baltimore (H.C.N., G.J.A.); the Division of Pulmonary Diseases, Children's Hospital, Denver (R.D., H.W.); the Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia (S.A., J.U.); and the Department of Dermatology, Kurume University, Fukuoka, Japan (T.H.). Presented in part at the 58th Annual Meeting of the Society for Investigative Dermatology, Washington, D.C., April 23–27, 1997.

Address reprint requests to Dr. Anhalt at the Division of Dermatoimmunology, Johns Hopkins University, 720 Rutland Ave., Room 771, Baltimore, MD 21205, or at ganhalt@jhmi.edu.

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Extract | Full Text  
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