Piebaldism

Last Updated: 2022-03-25

Author(s): Navarini

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.

Sun protection, camouflage