Very rare: < 1 / 20,000. Benign condition. Mutation of the c - kit gene (4q12) or of the SLUG gene (8q11.21) that results in a lack of melanocytes resulting in congenital leucodermia and poliosis. Presence of depigmented areas at the level of the forehead, trunk, chest, abdomen and extremities. The presence of a white blaze in the scalp hair (white forelock, typically of triangular shape) is the only manifestation of affection in 80 to 90 % of cases. The eyelashes and eyebrows can also be involved. Sometimes these areas contain hyperpigmented cafe-au-lait spots.
Differential diagnosis: Waardenburg syndrome, Fanconi anemia (see these terms)
Anesthetic implications:
none
References :
- Grob A, Grekin S.
Piebaldism in children.
Cutis 2016 ; 97 : 90-2.
- Oiso N, Fukai K, Kawada A, Suzuki T.
Piebaldism.
J Dermatol 2013 ; 40 : 330-5
Updated: December 2017