title: Association of urticaria pigmentosa with café-au-lait spots, neurofibromas and neurofibroma-like neoplasms: a mere coincidence?
abstract: BACKGROUND
  Urticaria pigmentosa (UP) is characterized by dense aggregates of mast cells in the dermis. There is consistent evidence from the literature that mast cells may play a pathogenetic role in the development of neurofibromas and other tumors.
  
  
  OBJECTIVE
  To study the concomitant appearance of UP with neurofibromas and neurofibroma-like neoplasms.
  
  
  METHODS
  We analyzed 31,752 records of patients examined at the Department of Dermatology in the year 2000, looking for UP and associated neurofibromas and neurofibroma-like neoplasms in persons younger than 18 years.
  
  
  RESULTS
  We identified a total of 27 patients suffering from UP, with 16 persons younger than 18 years. One 12-year-old Caucasian boy demonstrated multiple cutaneous mastocytomas consistent with the diagnosis of UP. On his trunk, four café-au-lait spots were found. A cutaneous neurofibroma was confirmed by skin biopsy. Magnetic resonance imaging detected multiple neoplasms located at the nerve roots of the spine, resembling plexiform neurofibromas.
  
  
  CONCLUSIONS
  There may be a concomitant appearance of UP and neoplasms, with mast cells possibly playing a causative role. The existence of neoplasms, including neurofibromas and neurofibroma-like lesions should be considered when examining UP cases.
authors.affiliation: Department of Dermatology and Allergy Biederstein, Technical University of Munich, Germany. m.moehrenschlager@lrz.tum.de
authors.forename: Matthias
authors.initials: M
authors.lastname: Möhrenschlager
authors.forename: Reinhard
authors.initials: R
authors.lastname: Engst
authors.forename: Stefan
authors.initials: S
authors.lastname: Müller-Weihrich
authors.forename: Walter
authors.initials: W
authors.lastname: Spiessl
authors.forename: Klaus
authors.initials: K
authors.lastname: Rüdisser
authors.forename: Lorenz B
authors.initials: LB
authors.lastname: Weigl
authors.forename: Berthold
authors.initials: B
authors.lastname: Jessberger
authors.forename: Markus
authors.initials: M
authors.lastname: Braun-Falco
authors.forename: Dietrich
authors.initials: D
authors.lastname: Abeck
authors.forename: Johannes
authors.initials: J
authors.lastname: Ring
mesh_terms: D000293:Adolescent; D001706:Biopsy; D019080:Cafe-au-Lait Spots / Q000150:complications* / Q000175:diagnosis; D002648:Child; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018318:Neurofibroma, Plexiform / Q000150:complications / Q000175:diagnosis; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D012878:Skin Neoplasms / Q000150:complications* / Q000175:diagnosis; D014582:Urticaria Pigmentosa / Q000150:complications* / Q000175:diagnosis