EPITHELIOMA ADENOIDES CYSTICUM: BASAL CELL NEVI, AGENESIS OF THE CORPUS CALLOSUM AND DENTAL CYSTS

Abstract

THE PRIMARY purpose of this paper is to present features of epithelioma adenoides cysticum which have not usually received adequate attention from dermatologists. From the earliest articles by Brooke 1 and others, 2 through that by Savatard 3 down to the most recent reports, the congenital defects associated with this disease have not been commented on or have received only brief mention. Recently we had the opportunity to observe a patient with lesions of epithelioma adenoides cysticum who also presented other developmental anomalies constituting a bizarre clinical picture. In addition to the cutaneous lesions there were partial agenesis of the corpus callosum (discovered at autopsy) and multiple dental follicular cysts. The latter proved to be the most serious condition, for a fibrosarcoma of the mandible appeared and metastases caused the patient's death. The patient also had mesodermal anomalies consisting of a bifid sixth rib and a fibroma of the ovary.