APLASIA CUTIS CONGENITA
By Gary M. White, MD
Aplasia cutis congenita (ACC) represents an absence of skin in utero. The scalp is most commonly affected. Often, the area has healed by delivery.
- Most cases of aplasia cutis congenita are unassociated with other conditions, but the following are rarely associated: trisomies (e.g., 10, 13); underlying skull defects and distal limb abnormalities (Adams-Oliver Syndrome); epidermolysis bullosa.
- Maternal exposure to methimazole has been reported to be associated with ACC in at least 19 cases, without a characteristic associated pattern.
- Histologic examination of the bullous variant shows the same picture as that found in meningoceles and encephaloceles, supporting the idea that bullous aplasia cutis may represent the forme fruste of a neural tube closure defect.
A congenital, well circumscribed area of alopecia of the scalp is most common. The skin is often atrophic ("membranous"). If a dense amount of hair is present at the periphery (hair collar sign), the possibility of an underlying meningocele or heterotopic brain tissue should be considered. In severe cases, the child may present with extensive scalp ulceration. Purse-string bunching of the skin around the ulcer may occur [Indian Dermatol Online J 2014;5:103-4].
Most lesions are small and do not require intervention. When older, the child may have the lesion removed for cosmesis.
Large, ulcerated lesions may be allowed to heal, although grafting may be done.
Courtesy O. Dale Collins, MD
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