Aplasia cutis congenita

Last Updated: 2019-08-27

Author(s): Anzengruber, Navarini

Congenital malformation of the skin on the head.

Incidence: 3/100 000.

Diaphragm eye ACC

Non-membrane ACC

Mostly spontaneous, rarely AD or AR.

There are several associated syndromes such as: Adam's Oliver syndrome, pyloric stenosis, Partau syndrome, etc.

Membrane ACC: Mostly circular hair/epidermis-less, rarely bullous, spot on the capillitium.

Non-membranous ACC: Larger and irregular hairless areas on the capillitium, which can extend to the nodes or meninges.

Clinic

No biopsy, especially with non-membrane ACC!

In non-membrane ACC, an MRI is necessary to detect the depth of the pathology. Only then should a cosmetic surgical procedure be planned, if necessary.

Capilitium

Meningitis

Sinus thrombosis 

In most cases, the epidermis is replicated in the course of time. In the case of larger defects, a cosmetic-surgical solution can be sought.

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