Bullous pemphigoid associated with mantle cell lymphoma.

Background: Bullous pemphigoid has developed in association with different types of malignant diseases, including a few cases of B-cell lymphoproliferative disorders. However, the paraneoplastic significance of this association is still controversial.

Observations: We describe a 39-year-old patient who presented with a bullous eruption and generalized lymphadenopathy. The results of histologic, immunofluorescence, and antigenic studies confirmed the diagnosis of bullous pemphigoid. The histopathologic and immunophenotypic features of a lymph node biopsy specimen were consistent with mantle cell lymphoma. There was total resolution of the mucocutaneous lesions when mantle cell lymphoma went into remission.

Conclusion: The age of the patient and the concomitant appearance and simultaneous remission of both diseases strongly suggest that bullous pemphigoid was a paraneoplastic phenomenon in the present case.