Aplasia cutis congenita - Hair and Scalp
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Synopsis

Aplasia cutis congenita is a congenital disorder seen in newborns. It typically manifests as an absence of the skin of the scalp but can occur in any location. While most children have no other associated abnormalities, cleft lip and palate, tracheoesophageal fistula, double cervix and uterus, patent ductus arteriosus, coarctation of the aorta, cutis marmorata telangiectatica congenita, AV fistulas, CNS dysraphisms, and intestinal lymphangiectasia have been reported with the anomaly. The newborn can present with open erosion or a healed, depressed scar.
Codes
ICD10CM:
Q84.8 – Other specified congenital malformations of integument
SNOMEDCT:
35484002 – Aplasia cutis congenita
Q84.8 – Other specified congenital malformations of integument
SNOMEDCT:
35484002 – Aplasia cutis congenita
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Differential Diagnosis & Pitfalls
Many cases of aplasia cutis congenita do not have other associated findings. However, the lesion has been associated with trisomy 13, which should be considered if other signs and symptoms of trisomy 13 are present.
Aplasia cutis congenita is also seen in focal dermal hypoplasia (Goltz syndrome), epidermolysis bullosa simplex, and defects in other genes affecting several other morphogenic pathways.
Also consider:
Aplasia cutis congenita is also seen in focal dermal hypoplasia (Goltz syndrome), epidermolysis bullosa simplex, and defects in other genes affecting several other morphogenic pathways.
Also consider:
- Erosions from herpes simplex virus (HSV)
- Ulceration from application of scalp electrodes or forceps
- Neonatal lupus
- Nevus sebaceus
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Last Updated:03/01/2015
Aplasia cutis congenita - Hair and Scalp
See also in: Overview