Acessibilidade / Reportar erro

Syndrome in question* * Work performed at the Dermatology Department - Unidade Local de Saúde Alto Minho, (ULSAM,EPE) – Viana do Castelo, Portugal.

Abstract

Neurofibromatosis is extremely variable in its presentation. Segmental neurofibromatosis (SNF), which corresponds to NF-type 5 in the Riccardi classification, is a rare disorder. It may go unrecognized if few lesions are observed. We present a case of segmental neurofibromatosis in a 28 year-old patient who presented with multiple papules and nodules distributed in dermatomal fashion on the trunk. The histopathological examination of the lesions revealed a non-encapsulated, well-circumscribed spindle cell neoplasm, which was consistent with neurofibromas.

Keywords:
Neurofibroma; Neurofibromatosis; segmentar neurofibromatosis

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