title: Multiple Café au Lait Spots in a Group of Fair-Skinned Children without Signs or Symptoms of Neurofibromatosis Type 1.
abstract: BACKGROUND
  The presence of six or more café au lait (CAL) spots is a criterion for the diagnosis of neurofibromatosis type 1 (NF-1). Children with multiple CAL spots are often referred to dermatologists for NF-1 screening. The objective of this case series is to characterize a subset of fair-complected children with red or blond hair and multiple feathery CAL spots who did not meet the criteria for NF-1 at the time of their last evaluation.
  
  
  METHODS
  We conducted a chart review of eight patients seen in our pediatric dermatology clinic who were previously identified as having multiple CAL spots and no other signs or symptoms of NF-1.
  
  
  RESULTS
  We describe eight patients ages 2 to 9 years old with multiple, irregular CAL spots with feathery borders and no other signs or symptoms of NF-1. Most of these patients had red or blond hair and were fair complected. All patients were evaluated in our pediatric dermatology clinic, some with a geneticist. The number of CAL spots per patient ranged from 5 to 15 (mean 9.4, median 9).
  
  
  CONCLUSIONS
  A subset of children, many with fair complexions and red or blond hair, has an increased number of feathery CAL spots and appears unlikely to develop NF-1, although genetic testing was not conducted. It is important to recognize the benign nature of CAL spots in these patients so that appropriate screening and follow-up recommendations may be made.
authors.affiliation: University of Massachusetts Medical School, Worcester, Massachusetts.
authors.forename: Jessica
authors.initials: J
authors.lastname: St John
authors.affiliation: Division of Dermatology, Department of Medicine, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts.
authors.forename: Heather
authors.initials: H
authors.lastname: Summe
authors.affiliation: Dermatology Professionals, North Attleboro, Massachusetts.|Department of Dermatology, Roger Williams Medical Center, Providence, Rhode Island.
authors.forename: Courtney
authors.initials: C
authors.lastname: Csikesz
authors.affiliation: Division of Dermatology, Department of Medicine, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts.|Division of Dermatology, Department of Pediatrics, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts.
authors.forename: Karen
authors.initials: K
authors.lastname: Wiss
authors.affiliation: Division of Genetics, Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts.
authors.forename: Beverly
authors.initials: B
authors.lastname: Hay
authors.affiliation: Division of Dermatology, Department of Medicine, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts. Leah.Belazarian@UMassMemorial.org.|Division of Dermatology, Department of Pediatrics, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts. Leah.Belazarian@UMassMemorial.org.
authors.forename: Leah
authors.initials: L
authors.lastname: Belazarian
mesh_terms: D000554:Ambulatory Care Facilities; D019080:Cafe-au-Lait Spots / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003880:Dermatology; D046169:Dermoscopy / Q000379:methods; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis*; D009885:Ophthalmology; D012494:Sampling Studies; D012720:Severity of Illness Index; D012880:Skin Pigmentation