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Case Reports
. 2014 Dec;31(6):410-2.
doi: 10.5114/pdia.2014.40942. Epub 2014 Dec 3.

Segmental neurofibromatosis

Michał Sobjanek  1 , Magdalena Dobosz-Kawałko  2 , Igor Michajłowski  1 , Rafał Pęksa  3 , Roman Nowicki  1
Affiliations
Case Reports

Segmental neurofibromatosis

Michał Sobjanek et al. Postepy Dermatol Alergol. 2014 Dec.

Abstract

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: mosaic-localized neurofibromatosis type 1; segmental neurofibromatosis; type V neurofibromatosis.

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Figures

Figure 1
Figure 1
Segmental neurofibromatosis: clinical features. Multiple, flesh colored, dome-shaped, soft to firm nodules situated in the right lumbar region
Figure 2
Figure 2
Neurofibroma: histopathologic features. Typical small spindled cells with “s-shaped” nuclei, vessels and small nerve fibers are visible
See this image and copyright information in PMC

References

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