Introduction:

neurofibromatosis is a genetic disorder that affects the growth of neural tissues, with an incidence of 1 in 4 000, with impact on life expectancy due its association with neoplasms and vascular disease. segmental neurofibromatosis is a subtype of neurofibromatosis type 1, with an approximate incidence of 1 in 20 000 to 25 000 people, it is characterized by skin lesions that affects a body segment without crossing midline, they generally have no family history or systemic involvement.

Clinical case:

a 63-year-old female patient with dermatosis affecting the posterior trunk unilaterally at the level of dermatomes t10-t11, characterized by multiple exophytic, dome-shaped, millimeter sized neoformations, soft in consistency and depressible on palpation. the histopatologic study of one of them confirmed the diagnosis of neurofibroma. the patient did not present neurological or ocular involvement, without affection in relatives. diagnosis of segmental neurofibromatosis was made.

Conclusions:

segmental neurofibromatosis is a rare pathology, although it is possibly underdiagnosed due its asymptomatic nature, which causes an apparent low incidence. patients may present variable systemic penetrance and a similar risk of neoplasm compared to patients with neurofibromatosis type 1. despite the benign nature reported in the literature, we suggest a multidisciplinary approach to patients.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )