Aplasia cutis congenita (ACC) is a rare congenital skin defect characterized by a focal or extensive absence of the epidermis, dermis, and occasionally subcutaneous tissue. The exact etiology of ACC is not well understood; however, it is likely due to impaired prenatal skin development. The disease presents as solitary or multiple lesions and can appear on any part of the body, although 70% to 90% of lesions are localized to the vertex of the scalp. ACC has been classified into six subtypes, some of which are associated with congenital dermatologic syndromes. Although most lesions are self-healing, certain locations and clinical characteristics should prompt a more thorough workup to screen for underlying soft tissue anomalies that can potentially be life-threatening.
A classification for ACC was proposed in 1986, which is still accepted today, and presented below.
Group 1: Scalp ACC without multiple anomalies
Group 2: Scalp ACC with limb abnormalities
Group 3: Scalp ACC with epidermal and organoid nevi
Group 4: ACC overlying congenital malformations
Group 5: ACC with associated fetus papyraceus or placental infarct
Group 6: ACC with epidermolysis bullosa
Group 7: ACC localized to extremities without blistering
Group 8: ACC due to specific teratogens
Group 9: ACC associated with malformation syndromes
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