1/20. Livedoid vasculopathy in a patient with factor v mutation (Leiden).BACKGROUND: Frequently, no underlying disease can be detected in patients with livedoid vasculopathy. For these forms, an unknown vaso-occlusive or thrombogenic process has been accused to play a role. Thus, a patient with livedoid vasculopathy was examined for different parameters which can be involved in coagulopathies. methods: Laboratory studies for different autoantigen reactive immunoglobulins, cryoglobulins, and circulating immune complexes were carried out. Besides dermatopathologic examination, a biopsy specimen was analyzed by direct immunofluorescence for immunoglobulin (Ig) and complement deposits. Furthermore, hemostaseological function tests including activated protein C (APC) resistance were undertaken. RESULTs: Positive only at very low titres were antinuclear antibodies and c-ANCA, all other parameters were within normal ranges or negative. Direct immunofluorescence revealed IgM, C3 and fibrogen deposits. Hemostaseological function tests demonstrated a pathologic activated protein c resistance and PCR analysis a heterozygous defect of the factor v (Leiden). CONCLUSIONS: The diagnosis of livedoid vasculopathy associated with factor v mutation (Leiden) was made. Since the underlying cause for livedoid vasculopathy often remains unknown, we suggest that hemostaseological function tests including APC resistance and factor v gene mutation analysis should be carried out. Further studies have to follow in order to elucidate the role of mutant factor v in livedoid vasculopathy and in cutaneous ulcerations.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
2/20. Livedoid vasculopathy associated with heterozygous protein c deficiency.Livedoid vasculopathy is characterized by recurrent painful ulceration of the feet, ankles and legs that heals with residual white atrophic scars. For many years, livedoid vasculopathy has been considered to be a primary vasculitic process. Recently, however, there has been a trend towards considering livedoid vasculopathy as an occlusive vasculopathy due to a hypercoagulable state. Livedoid vasculopathy (under the designation livedo vasculitis) was first reported to be associated with protein c deficiency in 1992. We describe an additional patient with livedoid vasculopathy associated with heterozygous protein c deficiency. This second reported case suggests that protein c deficiency may be one cause of the hypercoagulable condition in these patients and demonstrates the necessity for further investigation of thrombogenic factors underlying the disease.- - - - - - - - - - ranking = 0.8keywords = livedo (Clic here for more details about this article) |
3/20. calciphylaxis, a poorly understood clinical syndrome: three case reports and a review of the literature.Systemic calcification syndromes are a recognized complication occurring in some patients with end-stage renal disease (ESRD) and secondary hyperparathyroidism. These patients develop severe livedo reticularis and subcutaneous tissue lesions progressing to frank necrosis and ultimately large areas of eschar. Clinically this syndrome is known as calciphylaxis; these lesions are usually resistant to aggressive debridement, systemic antibiotics, and revascularization procedures. We report three patients with somewhat different clinical presentations but all sharing a common link of exquisitely painful leg ulcers initially being treated as ischemic lesions or venous stasis-type ulcerations. These three patients were diagnosed with calciphylaxis on the basis of clinical, biochemical, and histopathological criteria. Two patients underwent parathyroidectomy late in the progression of their disease, with some resolution of their ulcerative lesions.- - - - - - - - - - ranking = 0.78103275917078keywords = livedo reticularis, livedo, reticularis (Clic here for more details about this article) |
4/20. Livedo (livedoid) vasculitis and the factor v Leiden mutation: additional evidence for abnormal coagulation.We report the case of a patient with livedo vasculitis associated with the factor v Leiden mutation. This association provides additional support for abnormalities of coagulation in patients with this disorder. The spectrum of platelet, coagulation, and fibrinolytic disorders reported with livedo vasculitis is reviewed.- - - - - - - - - - ranking = 1.2keywords = livedo (Clic here for more details about this article) |
5/20. Response of livedoid vasculitis to intravenous immunoglobulin.Livedoid vasculitis is a chronic condition characterized by recurrent painful ulceration of the lower limbs, which heals to leave atrophie blanche surrounded by hyperpigmentation and telangiectasia. We report two patients with livedoid vasculitis who, after failure of conventional therapies, responded to intravenous immunoglobulin (IVIg). There was healing of areas of active ulceration and improvement of erythema, swelling and pain. IVIg has been used successfully to treat a variety of vasculitic disorders and appears to be well tolerated. We suggest that this treatment is offered to patients who have livedoid vasculitis that is unresponsive to other therapies.- - - - - - - - - - ranking = 1.2keywords = livedo (Clic here for more details about this article) |
6/20. Severe livedo vasculitis treated with potassium iodide.A young woman presenting severe livedo vasculitis (LV) was successfully treated with potassium iodide (KI). Previous attempts to cure her disorder with corticosteroids, low-dose aspirin, and azathioprine in various combinations had failed. To our knowledge, this is the first report of LV treated with KI.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
7/20. 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 = 1.6keywords = livedo (Clic here for more details about this article) |
8/20. Persistent cutaneous manifestations of hyperoxaluria after combined hepatorenal transplantation.A 30-year-old woman with primary hyperoxaluria type I (PHI) developed livedo reticularis with overlying ulcerations on her legs 16 months after receiving a liver-kidney transplant. A skin biopsy of the lesion showed deposits of calcium oxalate. To our knowledge, there have been no reported cases of livedo reticularis in patients with PH1 after a combined liver-kidney transplant.- - - - - - - - - - ranking = 1.5620655183416keywords = livedo reticularis, livedo, reticularis (Clic here for more details about this article) |
9/20. Idiopathic atrophie blanche.A 41-year-old woman presented with a 3-year history of purpuric lesions followed by superficial, painful ulcers and development of lesions on the lower legs and on the dorsa of the feet, particularly in the summer. The patient was asymptomatic during the winter months. On physical examination she had irregular, scleroatrophic, white-ivory, coalescent lesions on a livedoid basis, with purpuric and, in some lesions, pigmented borders with numerous telangiectatic capillaries. These lesions were localized on the medial sides of the lower legs and on the dorsa of the feet (Figure 1). Laboratory investigations were normal or negative, including complete blood cell count, platelets, coagulation indexes, erythrocyte sedimentation rate, serum immunoglobulins, antinuclear antibodies, anti-double-stranded dna, anticardiolipin, antiphospholipids, antineutrophilic cytoplasmic antibodies, circulating immunocomplexes, complement fractions (C3, C4), cryoglobulins, rheumatoid factor, and Rose-Waaler reaction. The only laboratory abnormality was an elevated fibrinogen level (472 mg/dL). Doppler velocimetry excluded a chronic venous insufficiency. Thoracic x-ray and abdominal ultrasound were normal. A digital photoplethysmograph revealed functional Raynaud's phenomenon. A biopsy specimen taken from a purpuric lesion showed an atrophic epidermis with parakeratosis and focal spongiosis. An increased number of small-sized vessels were observed within a sclerotic dermis. Most of the vessels in the upper dermis were dilated and showed endothelial swelling; some were occluded due to amorphous hyaline microthrombi (Figure 2). There were fibrinoid deposits around the vessels with thickening of the vessel walls. Extravasated erythrocytes were found throughout the upper and mid-dermis. There was a sparse perivascular lymphocytic infiltrate but no vasculitis. Direct immunofluorescence showed a perivascular microgranular deposit of IgM ( ), C3 ( ), and fibrinogen/fibrin ( ). On the basis of clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.- - - - - - - - - - ranking = 0.2keywords = livedo (Clic here for more details about this article) |
10/20. Sneddon's syndrome (livedo reticularis and cerebral thrombosis) with livedo vasculitis and anticardiolipin antibodies.A patient with widespread livedo reticularis and transient cerebral ischaemia (Sneddon's syndrome) is described. She also had painful scarring ulcers of the lower legs resembling livedo vasculitis and a circulating anticardiolipin antibody. We suggest that Sneddon's syndrome and livedo vasculitis may be pathogenetically related.- - - - - - - - - - ranking = 5.1051637958539keywords = livedo reticularis, livedo, reticularis (Clic here for more details about this article) |
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