Aplasia cutis congenita

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

In most cases, the epidermis is regenerated in the course of the procedure. For larger defects, a cosmetic surgical solution can be sought.

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2. Boisson B, Puel A, Picard C, Casanova JL. Human IκBα Gain of Function: a Severe and Syndromic Immunodeficiency. J Clin Immunol. 2017 Jul;37(5):397-412. doi: 10.1007/s10875-017-0400-z.
3. Perry BM, Maughan CB, Crosby MS, Hadenfeld SD. Aplasia cutis congenita type V: a case report and review of the literature. Int J Dermatol. 2017 Jun;56(6):e118-e121.