Koenen's tumor and angiofibromas: Epiloia

Abstract

A 39-year-old woman was attended for upper gastrointestinal bleeding, discovering gastroduodenal telangiectasis in an upper endoscopy; electrofulguration was performed with argon plasma. The physical examination revealed multiple facial angiofibroma and peri ungueal fibroma (Koenen’s tumors) of different sizes in the hands and feet. The CT scan revealed subependymal and periventricular calcifications in the brain, bilateral lung cystic lesions suggestive of linfangioleiomiomatosis, and multiple kidney angiolipomas. She had a history of episodic seizures since childhood, facial lesions, and intellectual incapacity, which constitutes the classic Vogt’s triad present in approximately 30% of patients with tuberose sclerosis, also known as Bourneville or epilogue syndrome. Two major criteria, or one major and two minor criteria, are needed to make the diagnosis. The patient had more than three major criteria.