Lymphadenopathy and entero-vesical fistula in Fabry's disease

A case of a 55-year-old man with known Fabry's disease, complicated by entero-vesical fistula and associated with enlarged mesenteric lymph nodes, is reported. Histopathologic and ultrastructural findings of the involved lymph nodes are described, and pathogenesis of the entero-vesical fistula is briefly discussed. This is the first case, to the authors' knowledge, of urologic complication in Fabry's disease.