eISSN: 2299-0046
ISSN: 1642-395X
Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii
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6/2014
vol. 31
 
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abstract:

Case report
Segmental neurofibromatosis

Michał Sobjanek
,
Magdalena Dobosz-Kawałko
,
Igor Michajłowski
,
Rafał Pęksa
,
Roman Nowicki

Postep Derm Alergol 2014; XXXI, 6: 410–412
DOI: https://doi.org/10.5114/pdia.2014.40942
Online publish date: 2014/12/03
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Segmental neurofibromatosis or type V neurofibromatosis is a rare genodermatosis characterized by neurofibromas, café-au-lait spots and neurofibromas limited to a circumscribed body region. The disease may be associated with systemic involvement and malignancies. The disorder has not been reported yet in the Polish medical literature. A 63-year-old Caucasian woman presented with a 20-year history of multiple, flesh colored, dome-shaped, soft to firm nodules situated in the right lumbar region. A histopathologic evaluation of three excised tumors revealed neurofibromas. No neurological and ophthalmologic symptoms of neurofibromatosis were diagnosed.
keywords:

segmental neurofibromatosis, type V neurofibromatosis, mosaic-localized neurofibromatosis type 1
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