Paraneoplastic pemphigus with autoantibody deposition in bronchial epithelium after autologous bone marrow transplantation.
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.
Fullerton
S H
SH
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Md.
Woodley
D T
DT
Smoller
B R
BR
Anhalt
G J
GJ
D001323:Autoantibodies / Q000032:analysis*; D016026:Bone Marrow Transplantation; D001980:Bronchi / Q000276:immunology*; D004848:Epithelium / Q000276:immunology; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000276:immunology*; D010392:Pemphigus / Q000276:immunology*; D013262:Stevens-Johnson Syndrome / Q000276:immunology
D001323:Autoantibodies