title: Sclerosing segmental neurofibromatosis.
abstract: Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait macules and/or neurofibromas limited to a single body segment. The neurofibromas in segmental neurofibromatosis are usually soft, non-tender nodules as in other types of neurofibromatosis. Histopathologically, they are usually non-encapsulated, loosely textured dermal tumors. We report a case of sclerosing segmental neurofibromatosis, in which the patient presented with several grouped, erythematous to brownish, firm tender nodules on the left side of the posterior neck. Histopathologically, the stroma was mostly very fibrotic, especially around hair follicles, in addition to the usual features of neurofibroma. The atypical clinical feature, hardness, and tenderness of the lesions may be associated with the fibrosis.
authors.affiliation: Department of Dermatology, Ajou University School of Medicine, Suwon 773-721, Korea.
authors.forename: Joong Sun
authors.initials: JS
authors.lastname: Lee
authors.forename: You Chan
authors.initials: YC
authors.lastname: Kim
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008875:Middle Aged; D009333:Neck / Q000473:pathology; D017253:Neurofibromatoses / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology*