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July 2009
By Kirkland Lau, DO, and Stephen M. Schleicher, MD
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CASE:
A 41-year-old woman is concerned about reddened, scaling spots that have been appearing on her forearms with increasing frequency in recent months. She reports that the lesions arise on areas that lost pigment years ago. Family history is significant for vitiligo, as well as for a distinctive white forelock of the frontal scalp. The patient’s mother, her two children, and a grandparent are affected.
WHAT IS YOUR DIAGNOSIS? |
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Piebaldism is a rare hereditary disorder characterized by a congenital white forelock and multiple symmetrical depigmented macules. Transmission occurs in an autosomal dominant manner and is the result of a mutation in the KIT proto-oncogene. Because of the loss of pigment, use of sunscreen is mandatory to help prevent occurrence of premalignant lesions, such as the actinic keratoses experienced by this patient. The disorder is not associated with systemic disease.
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Dr. Lau is an associate with a division of the DermDx Centers for Dermatology in Reading, Pennsylvania. Dr. Schleicher is director of the DermDOX Center in Hazleton, Pennsylvania, a clinical instructor of dermatology at the Philadelphia College of Osteopathic Medicine and Kings College in Wilkes-Barre, Pennsylvania, and an associate professor of medicine at the Commonwealth Medical School in Scranton, Pennsylvania. He is also a member of the EMERGENCY MEDICINE editorial board.
Emerg Med 41(7):27-28, 2009
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