RESUMO
Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions with unknown etiopathogenesis. We report a 16-year-old male patient who had been suffered from livedo reticularis for 10 years and multiple cerebral infarction for 2 years. Although the histologic finding of the skin did not show vasculitis and vascular occlusions, the patient revealed livedo reticularis and central nervous system symptoms. These findings were compatible with Sneddon's syndrome.
Assuntos
Adolescente , Humanos , Masculino , Sistema Nervoso Central , Infarto Cerebral , Livedo Reticular , Pele , VasculiteRESUMO
Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin. Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment. The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.