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| Gynecological Manifestation in Neurofibromatosis Type 1: Case Study and Review of the Literature
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M. ALAMI MERROUNI, I.ALILOU, K.M.SAOUD, N.MAMOUNI, S.ERRARHAY, C.BOUCHIKHI, A.BANANI
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Abstract:
Neurofibromatosis is a multivisceral genetic disease, frequent and of very variable severity. It is transmitted in the autosomal dominant mode. It affects the skin, bones and nervous system. There are at least seven kinds of neurofibromatosis depending on the causative gene. The most common is von Recklinghausen's neurofibromatosis, or NF1. It is due to the dysfunction of the NF1 gene located on chromosome 13, and coding for neurofibromin, a protein that protects against the development of tumours. NF2 is a bilateral acoustic neurofibromatosis, very rare and results in tumors on the auditory nerves, sometimes associated with other tumors of the central nervous system. We present here the case of a young girl suffering from neurofibromatosis with epilepsy and who presents a lesional process of the hemivulva and the pre-sacral space, and which is a dermatological manifestation of gynecological localization of neurofibromas. Management is not systematic and should be recommended only in the event of clinical manifestation and/or aesthetic discomfort with psychological repercussions.
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