We describe two nonconsanguineous white patients with multiple pigmented basal cell carcinomas (BCCs) that were histopathologically confirmed. The first patient had calcification of the cerebral falx, multiple keratocysts in the jaw, and other malformations of the cranial and finger bones. The second patient presented with multiple dermoid cysts, calcification of the cerebral falx, keratocysts of the mandible, and agenesis of left kidney. Both patients had palmar pits. Both denied any family history of cutaneous tumours. On dermatoscopic examination of patient 1, multiple, bluish, confluent and large globules were seen. The second patient had blue ovoid globules, arborizing vessels, and areas shaped like maple leaves and spoke-wheels. Based on the clinical and radiological features we diagnosed both patients as having Gorlin syndrome (GS). To our knowledge, there are no previous reports of white patients with GS showing only pigmented BCCs. The dermatoscopic patterns were different in the two patients, and to our knowledge, this is the first report of a patient with GS showing a spoked-wheel dermatoscopic pattern.
© The Author(s). CED © 2011 British Association of Dermatologists.