Información adicional
- Num_publicacion 65(5)
-
Resumen_ingles
Aplasia cutis congenita is a rare condition characterized by the absence of epidermis, dermis and, in some cases, subcutaneous tissues (skull bone or dura mater). It can appear anywhere on the body surface, although the most common location is the scalp, where an underlying bone defect is observed in 20% of the cases.
It is usually an isolated defect, although it can be accompanied by other malformations. While vascular, infectious, amniogenic and pharmacologic factors have been associated with aplasia cutis, its true etiology remains unclear.
In small lesions, even with an underlying bone defect, the prognosis is very good and treatment consists of hydration and protection of the affected area. However, in extensive lesions, with or without associated malformations, the prognosis varies widely due to the possibility of serious complications, such as meningitis or superior sagittal sinus hemorrhage. As these complications are potentially lethal, the therapeutic approach is controversial. The main objective is to prevent infection and hemorrhage. The most widely accepted option at the moment is surgical treatment, with skin and bone grafts, local rotation flaps or free flaps to close the defect, although successful results after the application of a conservative approach have recently been reported.
We present a case of extensive aplasia cutis congenita of the scalp with an underlying bone defect of nearly the same size, that was treated conservatively. To date, the response has been absolutely satisfactory.
- Palabras_clave_ingles Aplasia cutis congenita skin associated malformations conservative approach
- Todos_autores J. Pareja Grande, L. Olivares Sánchez, I. Sánchez Romero
- autores listados J. Pareja Grande, L. Olivares Sánchez, I. Sánchez Romero
-
Correspondecia
J. Pareja Grande. Servicio de Pediatría. 3.ª planta. Hospital «Santa Bárbara». Malagón, s/n. 13500 Puertollano (Ciudad Real).
Correo electrónico: jpareja100@hotmail.com - Titulo_ingles Aplasia cutis
- Centros_trabajo Servicio de Pediatría. Hospital «Santa Bárbara». Puertollano (Ciudad Real)
- Publicado en Acta Pediatr Esp. 2006; 64: 232-235
- copyright ©2006 Ediciones Mayo, S.A.
- Fecha recepcion 21/06/05
- Fecha aceptacion 29/06/05
Información adicional
- Num_publicacion 70(1)
-
Resumen_ingles
The epidermolysis bullosa constitute a group of determined genetically illnesses characterized by an excessive fragility of the skin to the friction forces. All of them take place for protein alterations, today known, that intervene in the union of the epidermis with the dermis. This leads to the formation of erosion and blisters, spontaneously or before a minimal traumatism. The patients suffering from this type of pathologies need a very careful clinical handling, which propitiates them the attention adapted to support a good quality of life, as well as the prevention and opportune treatment of the multiple complications that they can present, some of which can go so far as to threaten their life. We present of dystrophic epidermolysis bullosa case at birth, confirmed throughout histological and ultrastructural studies.
- Palabras_clave_ingles Epidermolysis bullosa congenital absence of skin clinical handling
- Todos_autores L. Blanquer Fagoaga, B. Garrido García, L. Martínez Casimiro, M. Romero Andreu
- autores listados L. Blanquer Fagoaga, B. Garrido García, L. Martínez Casimiro, M. Romero Andreu
-
Correspondecia
L. Blanquer Fagoaga. Servicio de Pediatría. Consorcio del Hospital General Universitario de Valencia. Avda. Tres Cruces, 2. 46014 Valencia.
Correo electrónico: laurblan@hotmail.com - Titulo_ingles Clinical handling of hereditary dystrophic epidermolysis bullosa: a case report
- Centros_trabajo Servicio de Pediatría. Consorcio del Hospital General Universitario de Valencia
- Publicado en Acta Pediatr Esp.2012; 70(1): 31-34
- copyright ©2012 Ediciones Mayo, S.A.
- Fecha recepcion 10/11/10
- Fecha aceptacion 23/11/10