Coexistence of aplasia cutis congenita, faun tail nevus and fetus papyraceus.

Background: Aplasia cutis congenita is a disorder of the skin embryonic development characterized by a defect of localized or widespread areas of skin at birth. The lesions are mostly oval, 1-3 cm in diameter, with localization on the parietal part of scalp (60%) and rarely on the face and extremities.

Main observations: Herein, we reported a case of aplasia cutis congenita termly born at 39 weeks of gestation to a 30-year-old mother with bronchial asthma attacks. She was referred for 3 punched-out punctate depressed defective lesions in 0.4 cm's diameter on the vertex covered with necrotic and hemorrhagic crusts. There was a hypertrichotic area consisting of tufts of terminal hair on the lumbosacral area over a sinus tract. Maternal perinatal drugs included aerosol salbutamol sulfate, ipratropium bromide and oral montelukast sodium for bronchial asthma. The pregnancy was firstly started as a di-chorionic, di-amniotic twin gestation, but deteriorated after the fetal resorption of the co-twin in the 20th gestational week resulting in fetus papyraceus.

Conclusion: In multi-gestational pregnancies, the presence of the fetus papyraceus or the death of the co-twins should make the neonatologists and dermatologists be aware of the possible cutaneous defects like aplasia cutis congenita. We emphasize that the possibility of this rare entity should be kept in mind in the presence of fetus papyraceus, perinatal drug use, maternal cigarette smoke, or maternal diseases like bronchial asthma in multiple gestations.