106. Lymphomatoid granulomatosis

The clinical findings in this patient initially suggested sarcoidosis, then leprosy. But after extensive study at two university medical centers and the Public Health Service Hospital at Carville, Louisiana, the final diagnosis was lymphomatoid granulomatosis—a disease with features resembling those of lymphoma and Wegener’s granulomatosis. Histologically, the characteristic finding is a granulomatous process with a polymorphous lymphoreticular infiltration, angiocentric and angiodestructive in nature, with atypical and occasionally bizarre lymphohistiocytic cells.

Although virtually any organ can be affected by this disease, pulmonary, neurologic, and cutaneous manifestations predominate. No consistently effective therapy has been established, and the prognosis is poor.

In the patient shown, the skin lesions resolved with corticosteroid therapy, but the neurologic difficulties worsened. And after a year of illness, the patient had become paraplegic. Curiously, no evidence of pulmonary disease ever emerged during that time.