Piebaldism

Last Updated: 2022-03-25

Author(s): Navarini A.

ICD11: -

Morgan, 1786

White forelock

  • Absence of melanocytes in lesional skin in well-defined localisation.
  • Classically only the skin is affected.

  • Born (forelock already objectifiable at birth, other areas also manifest later).
  • Associated with Klein-Waardenburg syndrome, ADFN syndrome, Tietz syndrome, Chediak-Higashi syndrome, Stargardt syndrome (irregular)

Genetic disease, autosomal dominant, mutation in KIT, SLUG genes.

Amelanotic macules, these are milky-white and irregularly bordered, sometimes also café-au-lait spots (with spherical melanomas).

Poliosis circumscripta (forelock, also eyebrows partially affected).

Hypomelanoe on the chin, dorsal neck, middle of the trunk, extremities (knees!).

  • Based on the typical clinic
  • Family history
  • Biopsy

Frontal (curl) as the most important symptom of the disease, trunk ventral may also be affected.

No melanin, no melanocytes in the lesions.

No healing to be expected.