[Segmental neurofibromatosis and germ-line mosaicism].
Segmental neurofibromatosis, also designated "NF-V" according to Riccardi's classification, should be considered a mosaic manifestation of one of the other types of neurofibromatosis, mostly of NF-I. Irrespective of the site and size of the body area affected, the patients are always at risk of germ-line mosaicism and they may therefore transmit the phenotype, but not the mosaic, to the next generation. Hence it follows that segmental neurofibromatosis does not constitute a distinct entity, and it is not reasonable to discriminate unilateral from bilateral or "hereditary" from "nonhereditary" forms.
Happle
R
R
Universitäts-Hautklinik Marburg.
D005854:Germ Cells; D006801:Humans; D009030:Mosaicism; D009456:Neurofibromatosis 1 / Q000145:classification / Q000235:genetics*
Segmentale Neurofibromatose und Keimbahnmosaik.