Aplasia cutis congenita (ACC) associated with fetus papyraceus is a rare subtype of aplasia cutis categorized as type V in Frieden's classification. It is characterized by stellate lesions in a symmetrical distribution over the trunk and proximal extremities. Conservative treatment is recommended, but there is not a well-defined therapeutic protocol. We report the case of a type V ACC in a preterm male newborn with lesions on the trunk and scalp successfully treated with topical 1% silver sulfadiazine and petrolatum gauze with an excellent evolution. This case associates a severe affectation of the scalp which represents a rare variant of type V ACC. K E Y W O R D S aplasia cutis congenita, treatment ACC, type V ACC 1 | INTRODUCTION Aplasia cutis congenita (ACC) is a rare disorder characterized by congenital absence of all skin layers in a localized or extensive area. 1 ACC is most frequently found as a solitary lesion on the scalp. However, face, trunk, buttocks and extremities might be affected. 1 It may present as an isolated defect, accompanied by other developmental abnormalities or within a syndrome. 2 Frieden classified ACC into nine groups based on the distribution of the affected area, associated anomalies, and mode of inheritance. 3
Presentación de casos clínicos RESUMEN La osteocondromatosis sinovial es una metaplasia benigna de la membrana sinovial que afecta a 1 de cada 100 000 personas, en su mayoría adultos, y es extremadamente infrecuente en edad pediátrica. Predomina en grandes articulaciones, sobre todo la rodilla, y la sintomatología es, por lo general, inespecífica. Dado que la radiografía simple no suele ser concluyente, se recurre a la resonancia magnética nuclear y a la tomografía axial computarizada para orientar el diagnóstico. Se expone el caso de una paciente de 10 años de edad con gonalgia y dismorfia en la patela izquierda de seis meses de evolución, con diagnóstico de osteocondromatosis sinovial. Se presenta el caso dado que se trata de una entidad muy rara en niños, pero que requiere un tratamiento quirúrgico precoz para evitar sus posibles complicaciones, como la destrucción articular progresiva o la malignización a condrosarcoma.
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