Paraneoplastic Pemphigus and Bronchiolitis Obliterans

What is it about?

Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome, is a rare disorder associated with underlying neoplasia. The common underlying neoplasms include non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, and Castleman’s disease. Though B-cell lymphoma is the most common underlying malignancy, only one case associated with splenic B-cell lymphoma has been recognized. The prognosis of PNP is very poor, and PNP-associated bronchiolitis obliterans (BO) is not uncommon.

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Photo by FlyD on Unsplash

Photo by FlyD on Unsplash

Why is it important?

We described a rare case of splenic B-cell lymphoma complicated with PNP and BO. The patient died 12 months later as a result of respiratory failure despite intensive immunosuppression and respiratory care. Consulting a dermatologist for sudden unusual polymorphic mucocutaneous manifestation is necessary and it may prompt work-up for possible underlying neoplasia. In addition, the association of PNP and BO must be kept in mind. If respiratory distress develops, it is mandatory to obtain quick and complete suppression of acute-phase inflammation to prevent severe bronchiolar inflammation that will progress to irreversible fibrosis and BO.