Diagnostic impact and sensitivity of skin biopsies in Sneddon's syndrome. A report of 15 cases

Background Sneddon's syndrome is defined as a combination of idiopathic liv edo racemosa generalisata and symptoms of cerebrovascular defect. The disea se usually starts with vascular symptoms in the epidermis, with neurologica l deficits becoming evident later. For this reason, histological examinatio n of skin biopsies and determination of arteriolar occlusion is of particul ar importance for reliable categorization and early diagnosis. To date, the se methods have been considered to be too insensitive. Objectives To evaluate the sensitivity of skin biopsies in Sneddon's syndro me. Methods We took a total of five deep punch biopsies (4 mm) from different a reas of the livedo (three from white and two from red areas) in 15 patients . Present knowledge of the pathogenic relationships and the particular anat omical features of the skin were fallen into account. Results The method had a sensitivity of 27% with one biopsy. 53% with two b iopsies and 80% with three biopsies taken from white areas in all cases. Conclusions Skin biopsies using the method presented achieved a high sensit ivity, suggesting that the diagnosis in clinically suspected cases could be confirmed in the majority of cases with this technique.