Recurrent massive subcutaneous hemorrhage in neurofibromatosis type 1: a case report

• Published in: Journal of Korean medical science Vol. 22; no. 4; pp. 728 - 730
• Main Authors: Baek, Sung Hoon, Kim, Ji Hye, Kim, Jun Sig, Han, Seung Baik, Cho, Jung Soo, Yoon, Yong Han, Kim, Lucia
• Format: Journal Article
• Language: English
• Published: Korea (South) The Korean Academy of Medical Sciences 01.08.2007
• Subjects: Case Report; Diagnosis, Differential; Female; Hematoma - etiology; Hematoma - pathology; Hemorrhage - etiology; Hemorrhage - pathology; Humans; Middle Aged; Neurofibromatosis 1 - complications; Neurofibromatosis 1 - pathology; Recurrence; Skin Diseases - etiology; Skin Diseases - pathology; Tomography, X-Ray Computed
• ISSN: 1011-8934; 1598-6357
• DOI: 10.3346/jkms.2007.22.4.728

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that has three major features: multiple neural tumors, cafe-au-lait spots, and pigmented iris hamartomas (Lisch nodules). The purpose of this case report is to advise physicians of the danger associated with the progression of fast-onset massive hemorrhage to hemodynamic instability, which mandates rapid treatment to prevent the development of a life-threatening condition. A 64-yr-old woman with NF-1 was admitted to the Emergency Department (ED) because of a rapidly growing, 10 x 5 x 3 cm-sized mass on the left back area. She had previously undergone surgery for a large subcutaneous hematoma, which had developed on her right back area 30 yr before. She became hemodynamically unstable with hypotension during the next 3 hr after admission to ED. Resuscitation and blood transfusion were done, and the hematoma was surgically removed. The mass presented as a subcutaneous, massive hematoma with pathologic findings of neurofibroma. We report a case of NF-1 that presented as recurrent, massive, subcutaneous hemorrhage on the back region combined with hypovolemic shock.