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ABSTRACT Aplasia cutis congenita (ACC) is a rare developmental malformation characterized by the absence of skin, extending to bone or dura in a localized or widespread area at birth. Mostly it’s seen in the scalp with an autosomal dominant inheritance and slight female predisposition. Generally, it is found as a solitary lesion without other anomalies, but sometimes it is reported associated with other syndromes. The association is much appreciated in case of malformations like Patau syndrome, Wolf-Hirschhorn Syndrome, Johanson- Blizzard Syndrome etc. It is not commonly seen with Edward Syndrome. Normally Edward syndrome is a rare entity and its association with ACC makes it very rare. As the survival rate of Edward syndrome is very low, these types of associations are unavailable in the literature. We present this very rare association and which was never reported before in my country.
Case Reports in Surgery
Aplasia Cutis Congenita of the Scalp with a Familial Pattern2016 •
Aplasia Cutis Congenita (ACC) is a condition characterized by congenital absence of skin, usually on the scalp. ACC can occur as an isolated condition or in the presence of other congenital anomalies. Here we describe a case of a 16-day-old baby girl with an isolated ACC of the scalp. Her elder two siblings have been diagnosed with ACC with concomitant cardiac or limb anomalies. The patient was managed conservatively until the defect has formed scar tissue 6 months later.
Aplasia cutis congenita is a rare malformation characterized by localized congenital absence of the skin. It rarely occurs on the trunk and limbs, and can occur in isolation or as part of a heterogeneous group of syndromes. We report a case of a 4-day-old boy with a 5.6-cm-diameter tumor, with a central crust, non-indurate and no inflammatory rim; localized on the scalp and a small, atrophic hairless scar appeared 6 months later (approximately 5cm in length) at the site of the previous tumor.
The Turkish journal of pediatrics
Aplasia cutis congenita: three cases with three different underlying etiologiesAplasia cutis congenita (ACC) is an uncommon condition in which localized or widespread areas of skin are absent or scarred at birth. There is no single underlying cause of ACC, as it simply represents a physical finding that reflects a disruption of intrauterine skin development. Here we report three cases of ACC of the scalp with three different etiologies: congenital rubella syndrome, trisomy 13 and fetal valproate syndrome. The aim of the present report is to increase awareness of these skin defects and emphasize the importance of underlying etiologies.
Anais Brasileiros de Dermatologia
Aplasia cutis congenita of the scalp- what are the steps to be followed? Case report and review of the literature2015 •
Journal of Obstetrics, Gynecology and Cancer Research
Extensive Aplasia Cutis Congenita: A Case ReportAplasia cutis congenita (ACC) is a rare congenital disorder characterized by localized or widespread skin absence. The most common site is the scalp and the main cause is unknown. Here we report a case of extensive ACC with scalp involvement. A female infant with an Apgar score of 9 and weighing 3406 gr was born by cesarean section in Besat Hospital, Sanandaj, Iran. A lesion measuring 5×10 cm with highly prominent vessels was on the forehead and parietal bone without scalp, skull, or bone tissue leading to the brain tissue being covered with a thin membrane. Other clinical examinations were normal. The newborn suffered from a ruptured aneurysm and intracerebral hemorrhage and underwent pharmacologic treatment. She died after 21 days of hospitalization. In order to prevent mortality in infants with skin defects, infection control is a priority. Antibiotics could be administered in cases with extensive lesions to prevent neonatal sepsis and improve scar tissue formation. Moreover, restorative measures should be taken if necessary.
New Emirates Medical Journal
Approach in Management of Aplasia Cutis Congenita in a Newborn with Adam Oliver SyndromeArquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery
First Case Report of an Infant with Aplasia Cutis Congenita of Scalp and Myelomeningocele2016 •
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