Category | Body area affected | Associated abnormalities | Inheritance |
Group 1: | |||
Scalp ACC without multiple anomalies | Scalp, usually vertex | Cleft lip and palate; tracheoesophageal fistula; double cervix and uterus; patent ductus arteriosus; omphalocele; polycystic kidney; mental retardation; cutis marmorata telangiectatica congenita | Autosomal dominant or sporadic |
Group 2: | |||
Scalp ACC with associated limb abnormalities | Midline scalp | Limb reduction abnormalities; two to three syndactyly; clubfoot; nail absence or dystrophy; skin tags on toes; persistent cutis marmorata; encephalocele; woolly hair; hemangioma; heart disease; cryptorchidism; postaxial polydactyly (one family) | Autosomal dominant |
Group 3: | |||
Scalp ACC with associated epidermal and organoid nevi | Scalp, may be asymmetric | Corneal opacities; scleral dermoids; eyelid colobomas; psychomotor retardation; seizures | Sporadic |
Group 4: | |||
ACC overlying embryologic malformations | Abdomen, lumbar skin, scalp; any site | Meningomyeloceles; spinal dysraphia; cranial stenosis; congenital midline porencephaly; leptomeningeal angiomatosis; ectopia of ear; omphalocele; gastroschisis | Depends on underlying condition |
Group 5: | |||
ACC with associated fetus papyraceus or placental infarcts | Multiple, symmetric areas, often stellate or linear, on scalp, chest, flanks, axillae, and extremities | Single umbilical artery; developmental delay; spastic paralysis; nail dystrophy; clubbed hands and feet; amniotic bands | Sporadic |
Group 6: ACC associated with EB: | |||
Blistering, usually localized, without multiple congenital anomalies | Extremities | Blistering of skin and/or mucous membranes; absent or deformed nails; metatarsus varus; congenital absence of kidney (seen in cases of recessive, dystrophic EB; dominant, dystrophic EB; and EB simplex) | Depends on EB type: may be autosomal dominant or recessive |
Widespread skin fragility with congenital anomalies | Large areas on extremities and torso | Pyloric or duodenal atresia; abnormal ears and nose; ureteral stenosis; renal abnormalities; arthrogryposis; amniotic bands; nail dystrophy | Autosomal recessive |
Group 7: | |||
ACC localized to extremities without blistering | Pretibial areas; dorsal aspects of hands and feet; extensor areas of wrists | None | Autosomal dominant or recessive |
Group 8: | |||
ACC caused by specific teratogens | Scalp (with methimazole); any area (with varicella and herpes simplex infections) | Imperforate anus (methimazole); signs of intrauterine infection with varicella and herpes simplex infections | Not inherited |
Group 9: | |||
ACC associated with malformation syndromes | Scalp; any location | Trisomy 13; 4p deletion syndrome; many ectodermal dysplasias; Johanson-Blizzard syndrome; focal dermal hypoplasia; amniotic band disruption complex; XY gonadal dysgenesis | Varies, depending on specific syndrome |
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