1/15. Oxalate kinetics and reversal of the complications after orthotopic liver transplantation in a patient with primary hyperoxalosis type 1 awaiting renal transplantation.We present the case of a young woman with end-stage renal disease secondary to primary hyperoxaluria type 1, who after 3 years and 6 months of maintenance hemodialysis, and despite intensification of the dialytic treatment, developed severe livedo reticularis in her extremities leading to ischemic cutaneous ulcerations, necessitating continuous intravenous infusion of narcotics for pain control. She received a liver transplant after native hepatectomy. However, due to positive crossmatch, she could not receive a kidney from that donor. After transplantation, following serial serum oxalate levels, the hemodialysis regimen was safely reduced from 4 h daily to 3 h three times weekly. Over the course of 6 weeks after liver transplantation, her livedo reticularis resolved, the ischemic ulcers markedly improved, she was weaned off all pain medications, and her erythropoietin-resistant anemia resolved. Our results suggest that in patients with primary hyperoxaluria type 1, who have received a liver transplant and are on maintenance hemodialysis, after serial serum oxalate determinations, some may safely be changed to a thrice-weekly maintenance hemodialysis regimen. Moreover, with this regimen the complications of systemic oxalosis can reverse.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
2/15. Familial primary cryofibrinogenemia.BACKGROUND: To our best knowledge this is the second case ever described of familial primary cryofibrinogenemia (CFG). patients: A 29-year-old Moroccan female and two of her three children suffered from painful purpura, slow healing small ulcerations and edema of both feet during the winter season. Laboratory investigations revealed the presence of cryofibrinogen in their blood plasma. All three patients were otherwise healthy and no associated disease could be demonstrated. CONCLUSIONS: The diagnosis of CFG has to be considered in patients with livedo reticularis, edema, painful purpura and slow healing ulcera after cold exposure. Cryofibrinogen-precipitates in the blood plasma have to be determined. Because secondary CFG occurs much more frequently than the primary form, it is important to rule out associated diseases through extensive physical examination and laboratory investigations. This communication also stresses the importance of a through family history of patients with CFG. An autosomal dominant mode of inheritance is supposed.- - - - - - - - - - ranking = 0.5keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
3/15. amantadine-induced peripheral neuropathy.We report a 48-year-old woman with a 17-year history of PD who developed a peripheral sensory-motor neuropathy secondary to chronic administration (8 years) of amantadine. Discontinuation of amantadine resulted in resolution of trophic skin ulcers, paresthesias, and distal weakness. amantadine may be hazardous to patients with severe and chronic livedo reticularis.- - - - - - - - - - ranking = 0.5keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
4/15. Use of intravenous immunoglobulin in livedo vasculitis.We describe a 36-year-old woman with livedo vasculitis (atrophie blanche) lasting for 15 years. After the failure of numerous therapeutic modalities the patient was treated with intravenous immunoglobulin. Significant resolution of the cutaneous lesions was seen with a concomitant alleviation of pain. To the best of our knowledge this is the first patient with livedo vasculitis described in the medical literature ever to be treated with IVIG and to achieve a successful outcome.- - - - - - - - - - ranking = 0.50025171046963keywords = livedo (Clic here for more details about this article) |
5/15. mixed connective tissue disease associated with skin defects of livedoid vasculitis.A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.- - - - - - - - - - ranking = 0.41687642539136keywords = livedo (Clic here for more details about this article) |
6/15. Five cases of livedo-like dermatitis (Nicolau's syndrome) due to bismuth salts and various other non-steroidal anti-inflammatory drugs.The authors report five cases of Nicolau's syndrome observed over a period of about 25 years. The disease had occurred after intramuscular injections of different drugs (bismuth, diclofenac and ibuprofen). In all the described cases the clinical aspect was characterized by a livedoid pattern followed by a slow necrotizing evolution with scar formation; in some cases surgical debridement and plastic reconstructive surgery were performed. In the past Nicolau's syndrome was described after intramuscular injections of bismuth salts for the treatment of syphilis; now, although still rare, it is described after injections of various aqueous drug solutions. The pathogenesis of Nicolau's dermatitis appears to be more complex than the previous hypothesized embolism caused by oleous drugs.- - - - - - - - - - ranking = 0.41687642539136keywords = livedo (Clic here for more details about this article) |
7/15. calciphylaxis with peau d'orange induration and absence of classical features of purpura, livedo reticularis and ulcers.calciphylaxis is an ill-defined syndrome that is commonly associated with chronic renal failure. Its heterogeneous clinical features include painful livedo reticularis-like purpuric patches and plaques, vesicles, irregularly shaped ulcers, and black eschars. Despite demonstration of extensive vascular arteriolar calcification in this syndrome, its exact pathogenesis remains unknown. Here, we report a case of calciphylaxis presenting with indurated plaques without the usual clinical picture of livedo reticulate purpura, ulcers or necrotic eschars. This case provides an opportunity to review the clinical spectrum of calciphylaxis and to discuss the therapeutic approaches and pathogenesis of this syndrome from deep intra-wall vascular calcification to the resulting infarctions of adjacent tissues.- - - - - - - - - - ranking = 2.5833752850783keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
8/15. Vasculopathy in von Recklinghausen's neurofibromatosis--a diagnostic quandary.Vasculopathy is a not well-described presentation of neurofibromatosis type 1 in the dermatologic literature. We report a fatal case of neurofibromatosis vasculopathy in a 28-year-old woman who presented with skin ulcers and livedo racemosa; extensive gastrointestinal ulcers; and infarction of the bowel, liver, and spleen. Being alert to this manifestation may avoid unnecessary delay in diagnosis and enable, whenever possible, appropriate therapy.- - - - - - - - - - ranking = 0.083375285078271keywords = livedo (Clic here for more details about this article) |
9/15. Embolia cutis medicamentosa.Embolia cutis medicamentosa or Nicolau syndrome is an acute necrotic condition of skin and subcutis that follows intramuscular administration of certain medicines. A young lady from rural area presented with an irregular-contoured ulceration with livedoid features on left deltoid region which she developed after receiving intramuscular injection of diclofenac sodium. The histopathological examination of the biopsied tissue demonstrated dead keratinocytes with degenerated epidermis and dermis. The condition improved on local management of wound with a residual scar.- - - - - - - - - - ranking = 0.083375285078271keywords = livedo (Clic here for more details about this article) |
10/15. Surgical treatment of livedo vasculitis.Surgical treatment for livedo vasculitis as a primary approach is not mentioned in the literature. A young female patient suffering from livedo vasculitis (atrophie blanche) was admitted with huge ulcerations and pre-ulcerative, infarcted skin areas of both feet and ankles. The lesions were promptly excised and skin grafted with excellent results. The course of treatment, including several surgical procedures, is described.- - - - - - - - - - ranking = 0.50025171046963keywords = livedo (Clic here for more details about this article) |
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