Piebaldism Clinical Presentation

Updated: Apr 06, 2021
  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Presentation

History

Graft versus host disease may arise solely within an area affected by piebaldism; therefore, piebaldism-affected skin may be immunologically different from normal skin. [19]

Next:

Physical Examination

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. [20] 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. [21]

Previous