Sclerosing peritubular nodules: a hereditary renal abnormality in von Recklinghausen's disease

A previously unrecognized type of renal lesion characterized by the formation of multiple hamartomatous sclerosing peritubular nodules in the cortex is described. These lesions were hereditary, appearing in three members of a family, which included the mother and her two sons, in association with familial central von Recklinghausen's disease (bilateral acoustic schwannomas). The nodules originated as concentric peritubular proliferations of collagen producing spindle cells, which ultrastructurally showed myofibroblastic differentiation. Four stages of nodule development leading to progressive sclerosis and tubular destruction are described. In spite of widespread involvement of the cortex with numerous nodules, in none of the three patients was there any clinical manifestation of renal dysfunction or hypertension.