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 medica...

Bart's syndrome is characterized by aplasia cutis congenita and epidermolysis bullosa. We present the case of a newborn male who developed blisters on the mucous membranes and the skin following congenital localized absence of skin. Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final d...

Aplasia cutis congenita is the congenital absence of skin. The majority of these defects involve the vertex of the scalp in the area overlying the sagittal sinus. The larger defects are predisposed to sudden lethal hemorrhage and require urgent closure. Local rotational scalp flaps are recommended for closure of the larger defects because they provide the most reliable coverage, eliminate the r...

Six inbred persons (five males and one female) in three generations of a single family are reported as having simple congenital absence of skin on the upper or lower limbs or both. The data suggest an autosomal recessive pattern of inheritance for this apparently new clinical entity.

Aplasia cutis congenita is a heterogeneous group of conditions usually involving the scalp as well as any other part of the body and is associated with a number of other congenital anomalies. We report on a newborn male with almost complete absence of skin and subcutaneous tissue in association with choanal atresia, syndactyly, imperforate anus, pulmonary hypoplasia, and other anomalies. To our...