Aplasia Cutis Congenita of the Scalp
نویسندگان
چکیده
Dr. João Paulo Cavalcante de Almeida – Rua Paulo Morais 130 60175-175 Fortaleza CE Brasil. E-mail: [email protected] Aplasia cutis congenita (ACC) is an uncommon disorder that presents as a focal defect of the skin at birth, frequently involving the midline over the skull vertex (70%), but it may affect any region of the body. Since 1767, about 500 cases have been reported in medical literature. The skin lesions are quite variable, ranging from absence of skin to a complete lack of subcutaneous tissue, bone or even the dura. The ACC can be isolated or associated with other malformations in a genetic syndrome, like Adams-Oliver syndrome. There are no exact etiologies for ACC, but some conditions have been associated to it like intrauterine vascular ischemia, amniotic adherences and viral infections. Teratogenic medications like metimazole and misoprostol have been proposed as causes of ACC. There is no consensus about the ideal treatment for the ACC, which is probably related to the low prevalence and incidence of this condition. The treatment options are conservative management or surgical correction, based on the clinical picture presented by each patient.
منابع مشابه
Aplasia cutis congenita: a case report
Aplasia cutis congenita is the congenital absence of skin mostcommonly affecting the scalp. No definite etiology is available butmultiple causes such as intrauterine infection, fetal exposure tococaine, heroin, alcohol or antithyroid drugs, vascular disruption,genetic causes, syndromes and teratogens have been suggested.We present an infant with extensive aplasia cutis of the trunk andthigh. He...
متن کاملAplasia Cutis Congenita of the Scalp with a Familial Pattern: A Case Report
Aplasia Cutis Conginita (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 days 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 wa...
متن کاملAplasia Cutis Congenita (ACC) and Seizure in a Premature Neonate: Could It Be a New Neurocutaneous Syndrome?
One of the few conditions associated with skin ulceration in the neonatal period is aplasia cutis congenita (ACC). ACC or congenital absence of the skin is considered an uncommon anomaly. This malformation commonly appears on the scalp as a solitary lesion, though it can be seen in other surfaces of the body such as the trunk, limbs and face. ACC can be associated with other physical anomalies ...
متن کاملAplasia cutis congenita
The patient was a female neonate bornprematurely at 36 weeks of gestation by a Cesareansection. During pregnancy, the mother had nohistory of fever, drug use, or X-ray exposure.The mother was gravida 2 and had no history ofabortion. Her first child was healthy. There wasno record of birth trauma. The perinatal historywas negative for intrauterine trauma, the use ofantithyroid medication, or mis...
متن کاملAplasia cutis congenita: a case report.
Aplasia cutis congenita is a rare anomaly presenting with absence of skin. The most common site is the scalp. No definite etiology is available. Heredity is proposed with not much evidence. We present an instance with ACC occurring in both mother and son, suggesting a hereditary etiology.
متن کاملAplasia cutis congenita: a rare case with extensive symmetrically distributed lesions.
Aplasia cutis congenita is a rare developmental disorder of the skin of neonates, usually presenting as a solitary lesion over the scalp. We report an interesting presentation of AC along with the histopathological features in a neonate with extensive lesions over scalp as well as in bilaterally symmetrical areas over trunk and thighs; such symmetrical distributions being rarely reported.
متن کامل