A Sudden Death in a Patient with Neurofibromatosis.

Lee, Bong Woo; Seo, Joong Seok; Kwon, Tae Jung; Lee, Won Tae

Case Report

Korean Journal of Legal Medicine 2000;24(1):92-97.
Published online May 31, 2000.

A Sudden Death in a Patient with Neurofibromatosis.

Bong Woo Lee, Joong Seok Seo, Tae Jung Kwon, Won Tae Lee

Department of Forensic Medicine, National Institute of Scientific Investigation, Korea.

Abstract

Neurofibromatosis type1(von Recklinghausen's disease) is characterized by multiple skin tumors and abnormal cutaneous pigmentation(cafe au lait spots). Unlikely the solitary neurofibroma, those encountered in von Recklinghausen's disease may cause significant morbidity. The vasculopathy, although poorly recognized manifestation of von Recklinghausen's neurofibromatosis is a major cause of fatal cases. One of its most dramatic presentation is the spontaneous hemothorax. We report a case of Von Recklinghausen's disease with multiple neurofibromatosis accompanying intrathoracic hemorrhage due to vascular rupture, which is the first legal autopsy case in korea.

Key Words: Neurofibromatosis type1, hemothorax