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Piebaldism Clinical Presentation

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
Updated: Jun 21, 2016


Graft versus host disease may arise solely within an area affected by piebaldism; therefore, piebaldism-affected skin may be immunologically different from normal skin.[14]



The white forelock is evident in 80-90% of those affected. Both hair and skin in the central frontal scalp are permanently white from birth or when hair color first becomes apparent. Regression of the white forelock has been described.[15] The forelock and white skin may have a triangular shape.

The eyebrow and eyelash hair may also be affected, either continuously or discontinuously with the forelock.

White spots may be observed on the face, trunk, and extremities and tend to be symmetrical in distribution and irregular in shape. They represent a focal lack of melanocytes. This depigmented skin may show a narrow border of hyperpigmentation or island of pigmentation and has white hair that is otherwise normal emanating from it.

White patches of hair may be located other than frontally in some patients. The only pigmentation change of skin or hair may be a white forelock in some patients.

Congenital leukoderma suggests the need for evaluation of ocular, auditory, and/or neurologic abnormalities.[16]



Piebaldism is a rare autosomal dominant genetic disorder.

Contributor Information and Disclosures

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.


Michael D Fox, MD Attending Physician, Department of Emergency Medicine, Marin General Hospital

Michael D Fox, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Geriatrics Society, American Academy of Family Physicians, California Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Albert C Yan, MD Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine

Albert C Yan, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology, Society for Pediatric Dermatology, American Academy of Pediatrics

Disclosure: Nothing to disclose.

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Distinguished physician with mark of distinction, a white forelock that his father and grandfather also shared.
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