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Syndrome in question
Aristóteles Rosmaninho1, Sandrina Carvalho2, Elena Oujo1
1Unidade Local de Saúde do Alto Minho, 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.
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