1/4. Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/4. Nodular regenerative hyperplasia of the liver, crest syndrome and primary biliary cirrhosis: an overlap syndrome?Nodular regenerative hyperplasia of the liver (NRHL) has been found in association with collagen vascular diseases, after drug therapy, with autoimmune disease, and with a variety of haematological disorders. The association of NRHL with the syndrome of calcinosis cutis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia (crest syndrome) has only been reported on two previous occasions. The liver disease usually associated with crest syndrome is primary biliary cirrhosis (PBC) and recently nodular hyperplasia of the liver has been reported in patients with early stage PBC. We present a case of NRHL with crest syndrome and serological and biochemical features of PBC, a newly recognised overlap syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/4. Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings.Linear scleroderma en coup de sabre (LScs) is a rare disorder not infrequently associated with neurologic symptoms, notably epilepsy. However, histopathologic documentation of intracerebral lesions in LScs is very limited and the etiology of the central nervous system symptoms has therefore never been convincingly established. We describe a 27-year-old woman with LScs and a longstanding history of epilepsy. Radiographic studies demonstrated a focal, intraparenchymal lesion in the left frontal lobe directly subjacent to the area of scleroderma on the forehead and scalp. The resected cerebral lesion revealed localized band-like sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels. These findings suggest that LScs may represent a neurocutaneous syndrome of vascular dysplasia similar to the sturge-weber syndrome, rather than a localized form of collagen vascular disease, as suggested by some.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/4. pterygium inversum unguis-like changes in scleroderma. Report of four cases.During an 18-month period, four patients with scleroderma were found to have nail findings suggestive or pterygium inversum unguis, a recently described condition. In the same time period, two other examples of the disorder were seen. pterygium inversum unguis-like change may represent an additional nail sign in scleroderma. There are possible mechanisms for its development in patients with Raynaud's phenomenon, connective tissue diseases, or peripheral vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |