Aplasia cutis congenita is the most common congenital cicatricial alopecia

Cicatricial alopecia

The term cicatricial alopecia refers to a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. A variety of distributions are possible. In some cases, hair loss is gradual, without symptoms, and is unnoticed for long periods...

, and is a congenital focal absence of epidermis with or without evidence of other layers of the skin.

It can be associated with Johanson-Blizzard syndrome

Johanson-Blizzard syndrome

Johanson–Blizzard syndrome is a rare, sometimes fatal autosomal recessive multisystem congenital disorder featuring abnormal development of the pancreas, nose and scalp, with mental retardation, hearing loss and growth failure...

, Adams-Oliver syndrome

Adams-Oliver syndrome

Adams–Oliver syndrome is a rare congenital disorder characterized by defects of the scalp and cranium , transverse defects of the limbs, and mottling of the skin.- Characteristics :...

, trisomy 13, and Wolf-Hirschhorn syndrome

Wolf-Hirschhorn syndrome

Wolf–Hirschhorn syndrome , also known as chromosome deletion 4p syndrome, Pitt-Rogers-Danks syndrome or Pitt syndrome, was first described in 1961 by Americans Herbert L...

.
It is also seen with exposure to Methimazole

Methimazole

Methimazole is an antithyroid drug, and part of the thioamide group.-Indications:...

 in utero. This dermatological manifestation has been linked to Peptidase D haploinsufficiency and a deletion in Chromosome 19.

See also

• List of cutaneous conditions

a form of aplasia cutis is also seen in patau syndrome (trisomy 13)
en.wikipedia.org/wiki/patau_syndrome

External Links

• Aplasia cutis congenita (ACC) on emedicine