DefinitionThis section has been translated automatically.
Occurrence/EpidemiologyThis section has been translated automatically.
Segmental NF1 is much rarer than "generalized" NF1. It affects 1 in 36,000 people compared to 1 in 2,500 for the common form of NF1.
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EtiopathogenesisThis section has been translated automatically.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Symptomatology corresponding to peripheral neurofibromatosis (NF type I) with atypical distribution of clinical symptoms. Exclusive involvement of one or more dermatomes or arrangement in a mosaic pattern. Associations with Cobb syndrome, a cutaneomeningospinal angiomatosis have been described (Pascual-Castroviejo I et al. 2008).
TherapyThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Gerhards G et al (1992) Unilateral lentiginosis - a segmental neurofibromatosis without neurofibromas. Dermatologist 43: 491-495
- Happle R (1991) Segmental neurofibromatosis and germline mosaicism. Dermatologist 42: 739-740
- Leverkus M et al (2003) Multiple unilateral schwannomas: segmental neurofibromatosis type 2 or schwannomatosis? Br J Dermatol 148: 804-809
- Listernick R et al (2003) Segmental neurofibromatosis in childhood. Am J Med Genet 121A: 132-135.
- Nagaoka Y et al (2002) Bilateral segmental neurofibromatosis. Acta Derm Venereol 82: 219-220.
- Pascual-Castroviejo I et al (2008) Segmental neurofibromatosis type 1 (NF1) associated with Cobb syndrome: case report. Neuropediatrics 39:341-343.
- Rose I et al (1991) Bilateral segmental neurofibromatosis. Dermatologist 42: 770-773
- Toy B (2003) Segmental neurofibromatosis. Dermatol Online J 9: 26
Incoming links (5)Café-au-lait stain; Lentiginosis partiale unilaterale; Neurofibromatosis type iv; Neurofibromatosis type v; Speckled lentiginous nevus;
Outgoing links (5)Cobb syndrome; Mosaicism; Mutation; Neurofibromatosis (overview); Neurofibromatosis peripheral;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.