Lymphomatoid granulomatosis after immunosuppression for pemphigus

Lymphomatoid granulomatosis developed in a patient with pemphigus vulgaris who had been undergoing immunosuppressive therapy for three years. At autopsy, typical angiodestructive and angiocentric lesions with large lymphoid cells (immunoblasts) were found in the lungs and mediastinal nodes. Although a direct causal relationship between immunosuppression and lymphomatoid granulomatosis is lacking, the association of the two has been described. Immunoblastic sarcoma is also seen in immunosuppressed patients, and the role of the immunoblast in both of these disorders indicates that they may be related. This report suggests that, like immunoblastic sarcoma, lymphomatoid granulomatosis may develop under conditions of diminished immunologic surveillance, whether iatrogenic or naturally occurring.