Tratamiento con Pulsed Dye Laser de mancha de vino oporto: seguimiento a 10 años / Treatment with Pulsed Dye Laser of wine stain Porto: 10-year follow-up

Las manchas de vino de oporto son un tipo de malformación capilar que afecta del 0,3 al 0,5% de la población. Están presentes desde el nacimiento como máculas o placas eritematosas en la piel o mucosas. Sin tratamiento, las lesiones tienden a oscurecerse con la edad, tornándose rojizas o color púrpura, y pueden desarrollar engrosamiento nodular o un granuloma piógeno asociado. Los tratamientos con láser proporcionan mejoría mediante la destrucción selectiva de la vasculatura. Se han utilizado una variedad de láseres vasculares selectivos, pero el tratamiento de primera elección es el láser de colorante pulsado. Los mejores resultados se obtienen cuando el tratamiento es instaurado tempranamente. (AU)

Port wine stains are a type of vascular malformation that affects 0.3% to 0.5% of the population. They are present from birth as erythematous macules or plaques on the skin or mucous membranes. Without treatment, these lesions tend to darken with age, becoming reddish or purple and may develop nodular thickening or an associated pyogenic granuloma. Laser treatments might provide an improvement by selective destruction of the vasculature. A variety of selective vascular lásers may be employed, with the pulsed dye laser as the gold standard treatment. Better results are obtained when the treatment is established early. (AU)

Vascular nevi in children.

BACKGROUND: Vascular nevi are cutaneous anomalies of angiogenesis and vasculogenesis resulting in various different clinical presentations. AIM: The purpose of our descriptive study was to observe the various types of vascular nevi in children and their features. METHODS: A total of 4256 pediatric cases attending the dermatology OPD during the study period from August 2002 to August 2004 were screened for vascular nevi. RESULTS: Out of these, 19 children (0.44%) had vascular nevi-17 hemangiomas of infancy (HOIs) and 2 port-wine stains. The mean age of the affected children was 1.3 years (ranging from 2.5 months to 8 years). There were 13 girls and 6 boys. Seventeen (89.5%) patients had progressing lesions and two (10.5%) had non-involuting ones. A solitary lesion was seen in ten (52.6%) cases and two to five lesions were present in five (26.3%) cases. The nevi were distributed over multiple sites in seven (36.8%) cases, the head and neck in six (31.6%) cases, the chest and lower limb in two (10.5%) cases each, and the upper limbs and genitalia in one (5.3%) case each. Among the hemangiomas of infancy, 15 (88.2%) cases of superficial type and 2 (11.8%) cases of deep type were seen. The cutaneous complications included ulceration in four cases and infection in one. CONCLUSIONS: HOIs were the most common vascular nevi of childhood.

[Acquired port wine stain: report of two cases]

Acquired port-wine stain is a rare vascular lesion that mimics a congenital port-wine stain clinically and histologically, but is acquired after birth. A survey on more than 60 reported cases in the literature reveals that most of these cases are idiopathic, but some of the cases developed after physical or mechanical trauma, hormonal changes, chronic sun exposure, and medications [OCP, isotretinoin, etc]. The pathogenesis of acquired port-wine stain like its congenital counterpart is obscure, but is thought to be related to a functional deficit of perivascular sympathetic neural control after mechanical trauma or other possible factors. Laser is the first choice of treatment in both congenital and acquired port-wine stains. We, herein report two rare cases of acquired port-wine stains. Both of the cases presented at the age of 15 years. One of them was idiopathic and the other one developed after a mild electrical bum on her hand. The histopathological findings of their biopsy specimens were compatible with the diagnosis of port-wine stain

Recurrent local palmar peeling associated with port-wine stain

There are no reports of association of specific cutaneous conditions in patients with local recurrent palmar peeling. Herein, we report a case with characteristic clinical presentation of local recurrent palmar peeling associated with a port-wine stain lesion.To the best of our knowledge, that association has not been reported before. Interesting points in the presented case are unilateral occurrence of the peeling disorder and its limitation to the region of port-wine stain

Facomatosis pigmentovascular: a propósito de dos casos / Phakomatosis pigmentovascularis: two case reports

La Facomatosis Pigmentovascular (FPV) es una enfermedad congénita poco frecuente. Consiste en la asociación de lesiones cutáneas pigmentarias y vasculares. Se han reportado cerca de 200 casos desde 1947, en su mayoría japoneses. En Latinoamérica se han publicado sólo 26 casos. Presentamos dos casos de FPV. El primero corresponde a una FPV tipo IIa en un paciente de 12 años y sexo femenino que presenta desde el nacimiento un nevus flammeus que abarca cara, cuello, tronco y extremidad inferior derecha asociada a una mancha mongólica aberrante. El segundo caso corresponde a una FPV tipo IIIa en una paciente de 9 años de edad y sexo femenino que presenta, al nacer, un nevus flammeus en región preauricular y cervical derecha, asociada a un nevus de spilus y a un nevus acrómico en tronco. Se presentan estos dos casos dada su baja frecuencia y para discusión en el contexto de la nueva clasificación de la FPV.

Tumores de la línea media / Tumors of the media line

Los tumores y lesiones no tumorales localizadas en la línea media del cuerpo o en rafes deben alertar al dermatólogo y al pediatra para estudiar posibles malformaciones subyacentes. Estas lesiones pueden tener comunicación con cavidad craneal (encefalocele, meningoencefaloquistes). Cuando estas lesiones están sobre la región lumbosacra pueden ser marcadores cutáneos de médula anclada o de lipomielomeningocele. En el presente trabajo se describen quiste dermoide, glioma nasal, meningioma y otras lesiones congénitas de la línea media. Se analiza el manejo reciente de estas lesiones y el estudio de imágenes (TC, RNM). Es esencial estudiar precozmente estos tumores y las malformaciones para instaurar tratamiento precoz, para prevenir futuras complicaciones neurológicas