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.
Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.
Paraneoplastic pemphigus1 is an autoimmune disease that accompaniesan overt or occult neoplasm and causes blisters. It is characterizedby the presence of IgG autoantibodies that react against desmosomaland hemidesmosomal plakin proteins,2,3,4,5 desmosomal transmembraneproteins (desmogleins),6 and an unidentified 170-kd antigen.Blistering of stratified squamous epithelium results from acantholysis,the loss of cellcell adhesion, induced by pathogenicantibodies against the desmogleins.6 The most commonly associatedneoplasms are, in decreasing order of frequency, non-Hodgkin'slymphoma, chronic lymphocytic leukemia, Castleman's disease,thymoma, retroperitoneal sarcomas, and Waldenström's macroglobulinemia.
Progressive respiratory failure with clinical features of bronchiolitisobliterans is frequently the cause of . . . [Full Text of this Article]
Case Reports
Patient 1
Patient 2
Methods
Results
Discussion
Source Information
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 2327, 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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