Diagnosis of Tuberous Sclerosis

Abstract

<h3>To the Editor.—</h3> I would like to comment on the important article by Cassidy et al wherein they studied the families of patients with tuberous sclerosis (1983;249:1302). The term<i>adenoma sebaceum</i>is no longer used to describe the skin papules found in the tuberous sclerosis complex. Pringle, who coined the term, did not appreciate how large normal sebaceous glands can be on the center of the face and misinterpreted these papules as neoplasms of sebaceous glands.¹The histopathology of the facial and periungual lesions in the tuberous sclerosis complex consists of widely dilated vascular spaces and abnormalities of the fibroblasts. They are called "angiofibromas," not "adenoma sebaceum." Cassidy et al claim that the adenoma sebaceum and periungual fibromas (ie, angiofibromas) are diagnostic of the tuberous sclerosis complex when they state that patients with them have "unequivocal findings demonstrating tuberous sclerosis." However, many authors, including most recently Meigel and Ackerman,