Bullous Pemphigoid Associated With a Lymphoepithelial Cyst of the Pancreas
Bullous pemphigoid (BP) is an acquired, autoimmune, subepidermal blistering disorder. A possible paraneoplastic association has been suggested; however, debate remains regarding the precise relationship of these neoplasms with BP. We present a case of recalcitrant BP in a 67-year-old man with a pancreatic neoplasm that was found to be a lymphoepithelial cyst. Immunoperoxidase staining of the cyst demonstrated C3d along the basement membrane of the stratified squamous epithelium, suggesting that the BP may have involved the lymphoepithelial cyst itself. Shortly after excision of the cyst, BP rapidly resolved without any immunosuppressive treatment, raising the possibility that the immunologic process involving the lymphoepithelial cyst of the pancreas was the inciting factor for the patient’s cutaneous disease. Although rare, some cases of BP may be a paraneoplastic process. A thorough screening via patient history and directed laboratory studies may be warranted in recalcitrant cases.
Conclusion
Our case suggests that paraneoplastic BP is a genuine entity. Indeed, the primary tumor itself may be the immunologic stimulus in the development of BP. Recalcitrant BP should raise the question of a neoplastic process that is exposing the BP antigen. If a thorough review of systems accompanied by corroborating laboratory studies suggests a neoplastic process, the suspect lesion should be further evaluated and surgically excised if clinically indicated. Further evaluation of neoplasms with advanced staining methods may aid in establishing the causative nature of tumors in the development of BP.
Acknowledgments
We are grateful to John Stanley, MD, and Aimee Payne, MD (both from Philadelphia, Pennsylvania), for theirinsights into this case.
