1/10. 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 = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
2/10. 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 = 5.1468443624251keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
3/10. 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 = 1.1747548994012keywords = livedo (Clic here for more details about this article) |
4/10. skin eruptions associated with microscopic polyangiitis.microscopic polyangiitis (MPA) is a systemic vasculitis histologically characterized by small-vessel involvement. Antineutrophil cytoplasmic antibodies (especially anti-myeloperoxidase antibodies) (MPO-ANCA) are often positive in serum. Although the skin is affected in 20-70% of patients, the precise description has been limited. This retrospective study analyzed clinical manifestations in patients of MPA with skin eruptions. Ten patients with skin eruptions diagnosed as MPA according to Chapel Hill consensus criteria consisted of 6 men and 4 women aged from 38 to 80 years (62.1 /- 13.3). Clinical manifestations, laboratory data, and histological findings were examined. purpura and petechiae in 6 patients, livedo in 2 patients, and erythema in 7 patients, especially erythema on the hands andor fingers in 4 patients, were observed. Histological findings from the eruptions in 7 patients showed perivascular lymphocyte infiltration in the upper dermis in 4 patients, and infiltration of lymphocytes and a few neutrophils around small arteries in the middle to deep dermis in 2 patients and diffuse infiltration of histiocytes and lymphocytes in the middle dermis in 1 patient. Cutaneous involvements in MPA showed a wide spectrum of clinical and histological findings.- - - - - - - - - - ranking = 0.14684436242515keywords = livedo (Clic here for more details about this article) |
5/10. Cutaneous vasculitis as a presenting feature of multiple myeloma: a report of 2 cases.We report two male patients who presented with symmetrical, painful purpura that evolved into bullae and necrotic ulcers, predominantly on the extremities, over two months in spite of conventional therapy including oral steroids. Examination showed livedoid and purpuric patches with necrotic centers in starburst pattern over the extremities and buttocks. The first case also had similar lesions over the ears. The clinical presentation and the histopathological examination suggested a diagnosis of necrotizing leukocytoclastic vasculitis (LCV). blood testing ruled out connective tissue disease, hepatitis b or C infection or streptococcal infection as underlying cause of vasculitis. serum antinuclear factor, antineutrophilic cytoplasmic antibody and anticardiolipin anticoagulant were negative in both cases. cryoglobulins were positive in case 2. An incidental finding was raised serum proteins and globulins in case 2. Further investigations revealed M band on electrophoresis and features of multiple myeloma on bone marrow biopsy in both cases. These cases emphasize the importance of simple investigations like serum proteins in the evaluation of LCV.- - - - - - - - - - ranking = 0.14684436242515keywords = livedo (Clic here for more details about this article) |
6/10. quinidine-induced photosensitive livedo reticularis-like eruption.A 39-year-old white man who was taking quinidine developed a purpuric eruption in a photodistributed livedo reticularis-like pattern. This is the fourth reported case of a similar eruption in patients taking quinidine. Possible pathophysiologic mechanisms for nonthrombocytopenic drug-induced purpuras are discussed.- - - - - - - - - - ranking = 5keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
7/10. Vasculitis in Reiter's syndrome.A patient is described with both Reiter's syndrome and cutaneous vasculitis characterised by livedo reticularis. The development of the Reiter's syndrome immediately preceded the onset of vasculitis. It is suggested that Reiter's syndrome is a previously unrecognised potential cause of vasculitis in which infection may initiate the vasculitic reaction.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
8/10. livedo reticularis and purpura: presenting features in fulminant pneumococcal septicemia in an asplenic patient.We report a case of pneumococcal septicemia in an asplenic, otherwise healthy, 31-year-old patient. He presented with a viral prodrome and skin findings of purpura and livedo reticularis. blood cultures identified streptococcus pneumoniae. Rapid initiation of appropriate therapy resulted in resolution of the infection and clearing of all skin signs.- - - - - - - - - - ranking = 1.7629203646065keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
9/10. Allergic granulomatosis (Churg-Strauss) associated with cutaneous manifestations: report of two cases.Allergic granulomatosis (Churg-Strauss) is sometimes associated with cutaneous manifestations. Biopsies of these manifestations are characteristic enough to allow for the diagnosis of this disease through histological examination. Case 1 involved a 34-year-old man who developed a low-grade fever, blood eosinophilia, paralysis of the right oculomotor nerve, and a polymorphic eruption. In addition, he suffered from preceding asthma and paresthesia of the legs and eventually developed heart failure. The histological examination of a biopsy taken from a hemorrhagic bulla showed a dense perivascular infiltration of eosinophils in the upper dermis and basophilic degeneration of collagen fibers. Case 2 involved a 41-year-old man who was afflicted with urticarial erythemas, livedoid erythemas, preceding asthma, and blood eosinophilia. During the course of his illness, he also developed bilateral peroneal nerve paralysis and two small erythemato-bullous lesions. The histological examination of a urticarial lesion revealed an abundant eosinophil perivascular infiltration; examination of a nodule revealed basophilic degeneration with a granulomatous reaction. Clinical and laboratory studies strongly suggested the diagnosis of allergic granulomatosis in both of these cases. The diagnosis was also supported by a histological examination of the skin lesions.- - - - - - - - - - ranking = 0.14684436242515keywords = livedo (Clic here for more details about this article) |
10/10. Reticulate purpura, cryoglobulinaemia and livedo reticularis.Two patients with type I cryoglobulinaemia are described, both of whom presented with purpura in a reticulate distribution on the legs, resembling the pattern of physiological livedo. It appeared that increased cooling due to sluggish blood flow in areas of the skin corresponding to the blue areas of physiological livedo may have caused the localization of cryoprecipitate at these sites. To investigate the pathogenesis of the net-like pattern of purpura, three subjects with physiological livedo reticularis of the thighs were studied with a laser-Doppler velocimeter. In two subjects, mean blood flux in the blue areas was 21% (P < 0.01) and 26% (P < 0.02) lower than in adjacent white areas, whereas in the third there was no significant difference. The cutaneous features of cryoglobulinaemia, and the mechanism of the reticulate purpura in this condition, are discussed.- - - - - - - - - - ranking = 5.2936887248503keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
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