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Pediatr Dermatol. 2016 Sep;33(5):526-9. doi: 10.1111/pde.12936. Epub 2016 Jul 28.

Multiple Café au Lait Spots in a Group of Fair-Skinned Children without Signs or Symptoms of Neurofibromatosis Type 1.

Author information

1
University of Massachusetts Medical School, Worcester, Massachusetts.
2
Division of Dermatology, Department of Medicine, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts.
3
Dermatology Professionals, North Attleboro, Massachusetts.
4
Department of Dermatology, Roger Williams Medical Center, Providence, Rhode Island.
5
Division of Dermatology, Department of Pediatrics, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts.
6
Division of Genetics, Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts.
7
Division of Dermatology, Department of Medicine, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts. Leah.Belazarian@UMassMemorial.org.
8
Division of Dermatology, Department of Pediatrics, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts. Leah.Belazarian@UMassMemorial.org.

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).

CONCLUSION:

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.

PMID:
27469520
DOI:
10.1111/pde.12936
[Indexed for MEDLINE]

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