Case Reports in Surgery / 2016 / Article / Tab 1

Case Report

Aplasia Cutis Congenita of the Scalp with a Familial Pattern

Table 1

Classification for ACC.

⁢GroupAssociated anomaliesInheritance

1Scalp ACC without multiple anomaliesCleft lip and palate, tracheoesophageal fistula, patent ductus arteriosus, omphalocele, mental retardation, polycystic kidneysAutosomal dominant or sporadic
2Scalp ACC with limb abnormalitiesLimbs reduced, syndactyly, clubfoot, encephalocele, nail dystrophy or absence, persistent cutis marmorataAutosomal dominant
3Scalp ACC with skin/organoid neviEpidermal nevi, organoid nevi, corneal opacities, scleral dermoids, eyelid colobomas, mental retardation, seizuresSporadic
4ACC overlying embryologic malformationsMeningomyelocele, spinal dysraphia, cranial stenosis, leptomeningeal angiomatosis, gastroschisis, congenital midline porencephaly, ectopia of ear, omphaloceleDepends upon underlying condition
5ACC with fetus papyraceus or placental infarctsSingle umbilical artery spastic developmental delay, spastic paralysis, clubbed hands and feet, amniotic bandsSporadic
6ACC associated with epidermolysis bullosaBlistering of skin and/or mucous membranes, deformed nails, pyloric or duodenal atresia, abnormal ears and nose, ureteral stenosis, renal anomalies, amniotic bandsDepends upon type of epidermolysis bullosa
7ACC localized to extremities without blisteringNoneAutosomal dominant or recessive
8ACC caused by teratogensImperforate anus (methimazole), other signs of intrauterine infection with varicella or herpes simplexNot inherited
9ACC associated with congenital syndromesTrisomy 13, 4p-syndrome, ectodermal dysplasia, focal dermal hypoplasia, amniotic band disruption complex, XY gonadal dysgenesis, Johanson-Blizzard syndromeDepends upon syndrome

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