Piebaldism

Classification according to ICD-10
E70.3	Albinism
ICD-10 online (WHO version 2019)

White forelock in piebaldism

Indira Gandhi

The piebaldism , also known as albinism partialis (partial albinism ) is referred to, is a very rare autosomal - dominant inherited syndrome , which is the formation of a white forelock comes (Poliosis, in 90% of cases). There is also a circumscribed hypopigmentation of the skin ( leukoderma ), these are white spots ( maculae ), especially on the face, neck, front upper body and flanks. Within these depigmentations , spot-shaped hyperpigmentation can often be found.

The white forelock has a characteristic appearance; it is a hair heterochromia . It grows in the forehead area and its tip points downwards.

A causal therapy of piebaldism is not possible.

root cause

Piebaldism is caused by a mutation in the KIT gene , which is located on the long arm of chromosome 4 and codes for the KIT receptor . The KIT receptor plays a role in the migration of melanocytes in the development of the embryo ( embryogenesis ). This leads to a lack or a maldistribution of melanocytes in certain areas. So despite the misleading name, piebaldism is not albinism , but leucism .

variants

Variants of piebaldism are von Waardenburg syndrome (associated with a hearing impairment) and Wolf syndrome .

See also

• Check
• Albinism Deafness Syndrome

swell

• Bernard A. Cohen: Pediatric Dermatology . Urban & Fischer Verlag, 2nd edition 2007, p. 168. ISBN 978-3-437-24250-2
• H. Kerl among others: Histopathology of the skin. Springer-Verlag 2003, p. 536. ISBN 3-540-41901-2
• Alexander Meves: Dermatology intensive course . Urban & Fischer Verlag 2006, p. 370. ISBN 3-437-41162-4

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !