Abstract

Hair collar sign is a rare malformation of the scalp, often associated with impairment of the central nervous system such as encephaloceles, meningoceles, and heterotopic brain tissue. We describe the cases of two male neonates, with maternal history positive for viral infections in pregnancy and no associated malformations of the nervous system, but coexistence of cutaneous plan nevi.

Highlights

  • Aplasia cutis is a condition in which localized or generalized areas of skin are absent at birth

  • A ring of hypertrichosis around an area of aplasia cutis often set on the vertex, is called "hair collar sign" and raises suspicion of ectopic neural tissue and an underlying defect of the skull

  • The skin and the nervous system are both derived from ectoderm

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Summary

Introduction

Aplasia cutis is a condition in which localized or generalized areas of skin are absent at birth. A ring of hypertrichosis around an area of aplasia cutis often set on the vertex, is called "hair collar sign" and raises suspicion of ectopic neural tissue and an underlying defect of the skull It is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. The second neonate, first child, born from spontaneous delivery after normal pregnancy, gestational age 36 wks + 3 days, mother 39 years with negative complex TORCH antibodies, weight at birth 2.385 Kg, no other physical anomalies, showed circular scalp lesion on the vertex with 0.5 mm of diameter (Figure 2) and plan nevus on right leg. CT scan of the skull showed no abnormal communication between the skin and the brain The only anomaly he showed was a cutaneous plan nevus on the left thigh, of diameter 1×2 cm and irregular shape.

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