Aplasia cutis congenita

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: LC60

Congenital malformation of the skin on the head.

Incidence: 3/100 000.

Membranous ACC

Non-membranous ACC

Mostly spontaneous, rarely AD or AR.

There are associated syndromes such as: Adams-Oliver syndrome, pyloric stenosis, Partau syndrome etc.

Membranous ACC: Mostly circular hairless/epidermis-less, rarely bullous, area on the capillitium. 

Non-membranous ACC: Larger and more irregular hairless areas on the capillitium that may extend to the nodule or meninges.

Clinic

No biopsy, especially for non-membranous ACC!

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

Meningitis

Sinus thrombosis

 

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