1/14. Sneddon's syndrome: a cause of cognitive decline in young adults.OBJECTIVE: To review the clinical, psychometric, laboratory, and radiologic findings of 6 patients with Sneddon's syndrome (SS) who presented with cognitive dysfunction rather than stroke. DESIGN AND methods: Case series. All patients fulfilled were diagnosed as SS based on the co-occurrence of livedo racemosa and neurologic disease. patients presenting with clinical stroke were excluded. RESULTS: patients presented with cognitive complaints at an early age and all noted skin lesions from 6 months to 10 years before onset of cognitive symptoms. Associated systemic disorders included hypertension and seizures. Laboratory evidence of a hypercoagulable condition was identified in 4 of 6 cases. brain MRI scans demonstrated atrophy, especially in parieto-occipital regions and cerebral blood flow on brain SPECT scan was reduced in a similar distribution. CONCLUSION: patients with SS can develop dementia without antecedent clinical stroke. While the specific pathogenic mechanism of dementia in SS remains speculative, the disease predominantly injures brain tissue in vascular "watershed" territories.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
2/14. Dermatopathia pigmentosa reticularis: a report of a family demonstrating autosomal dominant inheritance.Dermatopathia pigmentosa reticularis is a rare heritable disorder consisting of a triad of cutaneous findings including reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. In addition to this triad, other variable features of dermatopathia pigmentosa reticularis have been described, including adermatoglyphia, hypohidrosis or hyperhidrosis, and palmoplantar hyperkeratosis. Only 10 cases have been described in the world literature to date, and no clear inheritance pattern has been demonstrated. We present a patient with the classic features of the dermatopathia pigmentosa reticularis triad, along with adermatoglyphia, hypohidrosis, and punctate hyperkeratosis of the palms and soles. A family pedigree demonstrates the autosomal dominant inheritance of dermatopathia pigmentosa reticularis.- - - - - - - - - - ranking = 64.586137632842keywords = reticularis (Clic here for more details about this article) |
3/14. Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen.We describe a new method for treating livedoid vasculopathy. The typical presentation of livedoid vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. We believe this to be a very promising new therapy for livedoid vasculopathy and to be worth further investigation.- - - - - - - - - - ranking = 8keywords = livedo (Clic here for more details about this article) |
4/14. Type 1 diabetes mellitus associated with livedo reticularis: case report and review of the literature.livedo reticularis (LR) has been associated with numerous systemic diseases. Its relationship to diabetes mellitus (DM), however, has been poorly characterized. We report a case of LR in a 17-year-old with type 1 DM.- - - - - - - - - - ranking = 79.050116260995keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
5/14. Dermatopathia pigmentosa reticularis.Dermatopathia pigmentosa reticularis is a rare disorder that presents as reticulate pigmentation distributed widely all over the cutaneous surface. Only nine patients with this disease have been described previously. Our patient had no fingernail and toenail prints, a striking finding also noted in some, but not all, of the other nine patients. Our patient also had a history of a seizure disorder and had two cutaneous neurofibromas, unlike the other patients with this disorder. However, there were no other findings, such as Lisch nodules of the iris, to suggest a diagnosis of von Recklinghausen's disease.- - - - - - - - - - ranking = 40.366336020526keywords = reticularis (Clic here for more details about this article) |
6/14. A family with Dowling Degos disease showing features of Kitamura's reticulate acropigmentation.We report a family showing a combination of features described in the two, rare genodermatoses, Dowling Degos disease and Kitamura's acropigmentatio reticularis.- - - - - - - - - - ranking = 8.0732672041052keywords = reticularis (Clic here for more details about this article) |
7/14. Pigmentatio reticularis faciei et colli with multiple epithelial cysts.Brown-black reticular pigmentation developed on the face and neck of a man. This was associated with severe seborrhea and the formation of multiple epithelial cysts on the trunk. We propose the term pigmentatio reticularis faciei et colli with multiple epithelial cysts as a suitable title for this disorder, and we hypothesize that these skin changes in this disorder are of nevoid origin. One similar case has been reported as "lentiginosis senilis" in the Japanese literature.- - - - - - - - - - ranking = 40.366336020526keywords = reticularis (Clic here for more details about this article) |
8/14. Sneddon's syndrome.A 24 year old woman presented with generalised livedo reticularis and progressive intellectual decline. A CT scan of her head showed multifocal low density areas, consistent with cerebral infarction. cerebral angiography revealed occlusive disease of major vessels, an arteriovenous malformation, and moya-moya type anastomoses. There was no other associated systemic illness. Sneddon's syndrome was the provisional diagnosis. Her older brother had the same skin condition and was also shown to have occlusive cerebrovascular disease. The pathogenesis and natural history of this uncommon disease are highlighted.- - - - - - - - - - ranking = 17.744212264222keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
9/14. Vascular ulcers in scleroderma.Seven patients with scleroderma and either livedo reticularis or atrophie blanche lesions had ulcers of the lower extremity. Livedoid vasculitis, periarteritis nodosa, or endarteritis obliterans lesions were associated with the ulcers. Five patients had livedoid vasculitis, one patient had associated lupus panniculitis, and one patient had rheumatoid arthritis. Four patients had elevated ESRs, four had positive antinuclear antibody tests, and two had positive tests for rheumatoid factor. patients with scleroderma and livedoid vasculitis or livedo reticularis and ulcers should be examined to rule out underlying vascular disease or endarteritis obliterans.- - - - - - - - - - ranking = 37.488424528445keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
10/14. livedo reticularis with ulcers and circulating immune complexes.livedo reticularis is a reticulate skin pattern that is idiopathic or associated with systemic disease. A 30-year-old white man with livedo reticularis, winter ulcerations, and a peripheral neuropathy is described. His only laboratory abnormality remains the presence of circulating immune complexes. Therapy of this patient's condition and the significance of these immune complexes are discussed.- - - - - - - - - - ranking = 58.110548284749keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
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