Bronchiolitis obliterans (BO) may occur in a patient with paraneoplastic pemphigus.
Clinical features:
(1) pemphigus involving skin and mucous membranes
(2) underlying tumor triggering the paraneoplastic pemphigus (malignant lymphoma, Castleman disease, etc)
(3) variable other autoimmune manifestations (myasthenia gravis, etc)
(4) dyspnea on exertion
(5) progressive obstructive lung disease
Laboratory findings:
(1) antibodies to envoplakin, epiplakin, periplakin, desmoplakin
(2) antibodies to pemphigus vulgaris antigen (Dsg3)
The chest X-ray may initially appear relatively normal.
A wedge biopsy of lung shows bronchiolitis obliterans.
If the bronchiolitis obliterans progresses then lung transplantation may be necessary in carefully selected patients.
