Constrictive bronchiolitis obliterans is rare, and the pathogenesis of the
disease often remains unknown. This study reports on the case of a 38 yr-ol
d female with constrictive bronchiolitis obliterans and paraneoplastic pemp
higus associated with malignant lymphoma. The patient developed progressive
obstructive lung disease. The chest radiograph shelved almost normal lungs
. Paraneoplastic pemphigus is a newly described syndrome in which patients
have autoantibodies binding to some epithelia, including in the respiratory
tract. The disease develops in association with non-Hodgkin's lymphomas or
other malignant neoplasms. The case presented here suggests that constrict
ive bronchiolitis obliterans associated with paraneoplastic pemphigus may b
e one of the facets of autoimmune responses in this context.