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Giornale Italiano di Dermatologia e Venereologia 2000 December;135(6):697-701

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Aplasia cutis congenita with twin fetus dead in utero

Di Lernia V., Garavelli L. *, Vancini A. **, Magnani C. **

Azienda Ospedaliera - Reggio Emilia Arcispedale Santa Maria Nuova Unità Operativa di Dermatologia, Dipartimento Medico I * Ambulatorio di Genetica ** Sezione di Neonatologia e Terapia Intensiva Neonatale Dipartimento Materno-Infantile


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A case of aplasia cutis with extensive involvement of the skin of knees in a neonate is reported. No family history of aplasia cutis was present. During pregnancy, an associated monozygotic co-twin died and was resorbed in utero. On the basis of the history, absence of familial cases and clinical features (symmetry, extension, localization) the patient was diagnosed as aplasia cutis congenita type V according to Frieden’s classification (aplasia cutis congenita associated with fetus papyraceus or dead fetus). Ischemic and thrombotic events in the placenta and fetus appear to be to the basis of this subtype of aplasia cutis. The passage of thromboplastic material, through intertwin vascular shunts would be able to cause chorionic thrombosis resulting in infarcts in the placenta and fetal organs, including skin in the surviving twin. In the present case, the skin defects healed after a few months with scars. No associated anomalies and malformations were discovered.

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