Abstract
Type 1 neurofibromatosis (NF-1), or Von Recklinghausen disease, is an autosomal dominant genetic disorder. It is characterized by the presence of multiple neurofibromas. Herein, we reported an interesting case of a particularly aggressive neurofibroma that resulted in lower extremity gigantism; this form of plexiform neurofibromas, called elephantiasis neuromatosa (EN) is rarely described. It usually affects the lower limbs and can be complicated by intractable pain and multiple ulcers.
Highlights
Clin Med Img Lib 2:047 Published: June 16, 2016. This is the case of a twenty-eight-year-old man, with no significant family history, known to have type 1 neurofibromatosis who presented to the outpatient clinic for a growing lesion on the left lower extremity
Plexiform neurofibromas are diagnostic of NF-1 and represent a major cause of morbidity and mortality
They are found in 30-50% of NF-1 patients, appear in childhood and carry a risk of malignancy estimated around 5-10% [2,3]
Summary
This is the case of a twenty-eight-year-old man, with no significant family history, known to have type 1 neurofibromatosis who presented to the outpatient clinic for a growing lesion on the left lower extremity. Neurofibromatosis, Neurofibromas, Elephantiasis neuromatosa, Genodermatoses, Genetic skin diseases *Correspondence: Haber R, Department of Dermatology, Hotel Dieu de France University Hospital; Faculty of Medicine, Saint Joseph University, Beirut Lebanon, Tel: +9613841940, E-mail: roger.haber@usj.net.edu.lb The mass appeared during puberty and increased in size progressively during the years, affecting the quality of life of the patient.
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