Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome characterized by lipomatous hamartomas ranging in size from a few millimeters to several centimeters and affecting the head. Ocular anomalies and a variable degree of mental retardation with or without convulsions are usually observed. This disorder should be distinguished from other mosaic neurocutaneous phenotypes such as Proteus syndrome, oculocerebrocutaneous syndrome, and nevus sebaceous syndrome. We report the clinicopathologic findings of a 4-year-old Brazilian girl affected by this syndrome and review the literature. To our best knowledge, this is the first documented case of encephalocraniocutaneous lipomatosis occurring sporadically in South America.
The prevalence of HL was similar to previous reports. However, all histoid patients were "de novo" cases, differing from published studies. Fusocellular macrophage transformation could be explained by the differences in cytoskeleton proteins expressed in histoid lesions in comparison to other leprosy variants, with emphasis on vimentin and smooth muscle actin.
Our study is the first, to our knowledge, to demonstrate the increased presence of M2-Ms in ALM compared with SSM. Our findings suggest that the increased M2-Ms in ALM are associated with the main histopathological features predictive of adverse prognosis in CM, as well as the presence of metastasis, and that these cells can be related to the aggressive behaviour seen in ALMs.
Tufted angioma is a rare, benign, cutaneous angiomatous proliferation. It is more common in children and is clinically characterized by red-purple painfull plaques, more common in trunk. When spontaneous regression does not occur, proposed treatments (conventional surgery, steroid therapy, interferon and laser) achieve little success. We describe a case of a 11-year-old girl with a tufted angioma located in the trunk treated with pulsed-dye laser, showing important relief of local pain, and small reduction in lesion size. We have been motivated to describe this case due to the small number of such reports in Brazil and by the attempt to treat it with pulsed-dye laser. Keywords: Hemangioma; Lasers; Therapeutics
Resumo: O angioma em tufos é quadro raro, benigno, de proliferação cutânea angiomatosa. Acomete mais crianças e clinicamente caracteriza-se por lesões em placas, vermelho-violáceas, dolorosas e mais freqüentes no tronco. Quando não regride espon
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.