Paraneoplastic pemphigus with autoantibody deposition in bronchial epithelium after autologous bone marrow transplantation
S. H. Fullerton, D. T. Woodley, B. R. Smoller and G. J. Anhalt
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Md.
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.
