title: Segmental neurofibromatosis: immunocytochemical analysis of cutaneous lesions.
abstract: Cutaneous lesions from three patients with segmental neurofibromatosis were evaluated. Routine histologic studies revealed the presence of redimentary neural structures within an abundant collagenous matrix. The majority of the cells in all three cases expressed S-100 protein, suggesting their identity as Schwann cells. The stromal component stained positively for fibronectin and type IV collagen; the latter indicated the presence of basement membrane material. Embedded in the tumor mass were glandular epithelial structures that stained with epithelial membrane antigen antibody. Staining for factor VIII-related antigen revealed vascular endothelium and multiple scattered mast cells. In one case strands of cells stained with antibodies to desmin, suggesting muscle cell differentiation. This case may represent a distinct subset of neurofibromas.
authors.affiliation: Department of Dermatology, Jefferson Medical College, Philadelphia, PA 19107.
authors.forename: S
authors.initials: S
authors.lastname: Jaakkola
authors.forename: P
authors.initials: P
authors.lastname: Muona
authors.forename: W D
authors.initials: WD
authors.lastname: James
authors.forename: M
authors.initials: M
authors.lastname: Hannuksela
authors.forename: J
authors.initials: J
authors.lastname: Karvonen
authors.forename: J
authors.initials: J
authors.lastname: Peltonen
authors.forename: J
authors.initials: J
authors.lastname: Uitto
mesh_terms: D000328:Adult; D000368:Aged; D002467:Cell Nucleus / Q000032:analysis; D003094:Collagen / Q000032:analysis; D003593:Cytoplasm / Q000032:analysis; D003893:Desmin / Q000032:analysis; D005260:Female; D005353:Fibronectins / Q000032:analysis; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D008875:Middle Aged; D018396:Mucin-1; D009456:Neurofibromatosis 1 / Q000032:analysis* / Q000473:pathology; D009418:S100 Proteins / Q000032:analysis; D012878:Skin Neoplasms / Q000032:analysis* / Q000473:pathology; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D003893:Desmin; D005353:Fibronectins; D008562:Membrane Glycoproteins; D018396:Mucin-1; D009418:S100 Proteins; D014841:von Willebrand Factor; D003094:Collagen