Paraneoplastic Pemphigus With Autoantibody Deposition in Bronchial Epithelium After Autologous Bone Marrow Transplantation

S. Honor Fullerton, David T. Woodley, Bruce R. Smoller, Grant J. Anhalt

Research output: Contribution to journalArticlepeer-review

Abstract

Paraneoplastic pemphigus is a newly described syndrome in which patients have a severe mucocutaneous eruption with clinical features reminiscent of both erythema multiforme major (Stevens-Johnson syndrome) and pemphigus vulgaris, in association with non-Hodgkin’s lymphomas and other malignant neoplasms. These patients have autoantibodies that bind to a characteristic set of epidermal proteins: desmoplakin I and desmoplakin II (molecular weight equals 250 kd and 210 kd, respectively), both major cytoskeletal structural proteins associated with desmosome cellular junctions within all epithelia, the bullous pemphigoid antigen, a 230 kd protein associated with hemidesmosomes, and a 190 000-d protein that has not been characterized. In this report, we describe a patient with paraneoplastic pemphigus who had (1) non-Hodgkin’s lymphoma in apparent complete remission following autologous bone marrow transplantation, (2) very tense blisters reminiscent of bullous pemphigoid, (3) a unique pattern of immune deposits within the skin, and (4) IgG deposits within the epithelium of the pulmonary bronchi associated with respiratory compromise.

Original languageEnglish (US)
Pages (from-to)1500-1502
Number of pages3
JournalJAMA: The Journal of the American Medical Association
Volume267
Issue number11
DOIs
StatePublished - Mar 18 1992

ASJC Scopus subject areas

  • Medicine(all)

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