The Experts below are selected from a list of 198 Experts worldwide ranked by ideXlab platform
Mark R. Pittelkow - One of the best experts on this subject based on the ideXlab platform.
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Homocysteinemia and Livedoid Vasculitis
Journal of the American Academy of Dermatology, 1999Co-Authors: Gillian E. Gibson, Mark R. PittelkowAbstract:Homocystinuria is a rare autosomal recessive genetic abnormality associated with significantly increased levels of homocysteine in the blood.1 The disorder is associated with arterial and venous thromboembolic events at an early age. Patients may have a marfanoid habitus, mental retardation, and psychiatric disorders. Skin manifestations include skin and hair hypopigmentation, a malar rash, livedo reticularis, and leg ulcers. Deficiencies of several enzymes involved in homocysteine metabolism, including cystathionine β-synthase and 5,10-methylenetetrahydrofolate reductase, are associated with homocystinuria. In the general population, an association between total plasma homocysteine levels and coronary artery disease, peripheral artery disease, stroke, and venous thrombosis has been shown in more than 75 clinical and epidemiologic studies.2,3 Livedoid Vasculitis is a cutaneous occlusive vasculopathy characterized by recurrent painful ulcers of the lower extremities in association with a persistent livedo reticularis (Fig 1). Histologic examination reveals segmental hyalinization and primarily noninflammatory thrombotic occlusion of dermal arterioles. Some patients with Livedoid Vasculitis have enhanced procoagulant or deficient fibrinolytic activity of plasma.4 Treatment with tissue plasminogen activator (t-PA) has promoted healing of chronic leg ulcers caused by Livedoid Vasculitis.5 No studies to date have addressed the possible contribution of increased blood homocysteine levels to the pathogenesis of small-vessel occlusive vasculopathy. It is possible that increased homocysteine levels alone or in combination with other defects of inherited thrombophilia (the “doublehit” effect) may be associated with an increased risk of small-vessel thrombosis. Klein and Pittelkow5 found a high incidence of anticardiolipin antibodies, lupus anticoagulant, increased levels of t-PA inhibitor, and low levels of endogenous t-PA activity in patients with Livedoid Vasculitis. An increased risk of large vessel thrombosis was also demonstrated in patients with homocystinuria who in addition had factor V Leiden.6 The aim of this study was to determine whether homocysteine levels are increased in patients with Livedoid Vasculitis and, if so, whether this is an isolated phenomenon or occurs in association with other inherited or acquired defects leading to thrombophilia.
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Antiphospholipid syndrome and the skin
Journal of the American Academy of Dermatology, 1997Co-Authors: Gillian E. Gibson, Mark R. PittelkowAbstract:The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for the syndrome are the lupus anticoagulant and anticardiolipin antibodies. Clinical features include recurrent thrombotic events (arterial or venous), repeated fetal loss, and thrombocytopenia. Cutaneous manifestations may occur as the first sign of antiphospholipid syndrome. These include livedo reticularis, necrotizing Vasculitis, Livedoid Vasculitis, thrombophlebitis, cutaneous ulceration and necrosis, erythematous macules, purpura, ecchymoses, painful skin nodules, and subungual splinter hemorrhages. Antiphospholipid syndrome may also be associated rarely with anetoderma, discoid lupus erythematosus, cutaneous T-cell lymphoma, or disorders that closely resemble Sneddon or Degos syndromes. Noninflammatory vascular thrombosis is the most frequent histopathologic feature observed. Prophylaxis and treatment of thrombosis in patients with antiphospholipid syndrome relies principally on anticoagulant and antiplatelet agents.
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Tissue Plasminogen Activator for Treatment of Livedoid Vasculitis
Mayo Clinic proceedings, 1992Co-Authors: Kenneth L. Klein, Mark R. PittelkowAbstract:Livedoid Vasculitis, a hyalinizing vasculopathy, is characterized by extensive formation of microthrombi and deposition of fibrin in the middermal vessels, which result in epidermal infarction, ulceration, and formation of stellate scars. In a prospective study of nonhealing ulcers in patients with Livedoid Vasculitis, we found a high incidence of anticardiolipin antibodies, lupus anticoagulants, increased levels of plasminogen activator inhibitor, and low levels of endogenous tissue plasminogen activator (t-PA) activity. This procoagulant tendency and decreased fibrinolysis may provide an explanation for the occlusive vasculopathy often noted in biopsy specimens from these patients. On the basis of these findings, we proposed that fibrinolysis with recombinant t-PA would lyse microvascular thrombi, restore circulation, and promote wound healing. In six patients who had nonhealing ulcers caused by Livedoid Vasculitis and in whom numerous conventional therapies had failed, low-dose t-PA (10 mg) was administered intravenously during a 4-hour period daily for 14 days. Five of the six patients had dramatic improvement; almost complete healing of the ulcers occurred during hospitalization, and tissue oxygenation, as measured by transcutaneous oximetry, increased. The one treatment failure was due to rethrombosis of the microvasculature; this patient was subsequently re-treated but with concurrent anticoagulation, and her leg ulcers healed. We conclude that daily administration of a low dose of t-PA is safe and effective treatment for nonhealing ulcers due to occlusive vasculopathy.
R. Russell-jones - One of the best experts on this subject based on the ideXlab platform.
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Response of Livedoid Vasculitis to intravenous immunoglobulin
The British journal of dermatology, 2002Co-Authors: F.e. Ravat, A.v. Evans, R. Russell-jonesAbstract: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.
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Livedoid Vasculitis: a manifestation of the antiphospholipid syndrome?
The British journal of dermatology, 1999Co-Authors: K. Acland, A. Darvay, S H Wakelin, R. Russell-jonesAbstract:Livedoid Vasculitis, otherwise known as segmental hyalinizing Vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a Vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with Livedoid Vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that Livedoid Vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition.
Gillian E. Gibson - One of the best experts on this subject based on the ideXlab platform.
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Homocysteinemia and Livedoid Vasculitis
Journal of the American Academy of Dermatology, 1999Co-Authors: Gillian E. Gibson, Mark R. PittelkowAbstract:Homocystinuria is a rare autosomal recessive genetic abnormality associated with significantly increased levels of homocysteine in the blood.1 The disorder is associated with arterial and venous thromboembolic events at an early age. Patients may have a marfanoid habitus, mental retardation, and psychiatric disorders. Skin manifestations include skin and hair hypopigmentation, a malar rash, livedo reticularis, and leg ulcers. Deficiencies of several enzymes involved in homocysteine metabolism, including cystathionine β-synthase and 5,10-methylenetetrahydrofolate reductase, are associated with homocystinuria. In the general population, an association between total plasma homocysteine levels and coronary artery disease, peripheral artery disease, stroke, and venous thrombosis has been shown in more than 75 clinical and epidemiologic studies.2,3 Livedoid Vasculitis is a cutaneous occlusive vasculopathy characterized by recurrent painful ulcers of the lower extremities in association with a persistent livedo reticularis (Fig 1). Histologic examination reveals segmental hyalinization and primarily noninflammatory thrombotic occlusion of dermal arterioles. Some patients with Livedoid Vasculitis have enhanced procoagulant or deficient fibrinolytic activity of plasma.4 Treatment with tissue plasminogen activator (t-PA) has promoted healing of chronic leg ulcers caused by Livedoid Vasculitis.5 No studies to date have addressed the possible contribution of increased blood homocysteine levels to the pathogenesis of small-vessel occlusive vasculopathy. It is possible that increased homocysteine levels alone or in combination with other defects of inherited thrombophilia (the “doublehit” effect) may be associated with an increased risk of small-vessel thrombosis. Klein and Pittelkow5 found a high incidence of anticardiolipin antibodies, lupus anticoagulant, increased levels of t-PA inhibitor, and low levels of endogenous t-PA activity in patients with Livedoid Vasculitis. An increased risk of large vessel thrombosis was also demonstrated in patients with homocystinuria who in addition had factor V Leiden.6 The aim of this study was to determine whether homocysteine levels are increased in patients with Livedoid Vasculitis and, if so, whether this is an isolated phenomenon or occurs in association with other inherited or acquired defects leading to thrombophilia.
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Antiphospholipid syndrome and the skin
Journal of the American Academy of Dermatology, 1997Co-Authors: Gillian E. Gibson, Mark R. PittelkowAbstract:The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for the syndrome are the lupus anticoagulant and anticardiolipin antibodies. Clinical features include recurrent thrombotic events (arterial or venous), repeated fetal loss, and thrombocytopenia. Cutaneous manifestations may occur as the first sign of antiphospholipid syndrome. These include livedo reticularis, necrotizing Vasculitis, Livedoid Vasculitis, thrombophlebitis, cutaneous ulceration and necrosis, erythematous macules, purpura, ecchymoses, painful skin nodules, and subungual splinter hemorrhages. Antiphospholipid syndrome may also be associated rarely with anetoderma, discoid lupus erythematosus, cutaneous T-cell lymphoma, or disorders that closely resemble Sneddon or Degos syndromes. Noninflammatory vascular thrombosis is the most frequent histopathologic feature observed. Prophylaxis and treatment of thrombosis in patients with antiphospholipid syndrome relies principally on anticoagulant and antiplatelet agents.
Edward J. O'keefe - One of the best experts on this subject based on the ideXlab platform.
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Failure of Livedoid Vasculitis to Respond to Tissue Plasminogen Activator
Archives of dermatology, 1995Co-Authors: Dedee F. Murrell, Joseph Jensen, Edward J. O'keefeAbstract:Recently, low-dose intravenous recombinant tissue plasminogen activator (t-PA) was reported as a safe and effective treatment for refractory Livedoid Vasculitis, 1 also known as atrophie blanche or PURPLE. (Milstone et al 2 proposed a unifying terminology for the multiple presentations of patients with the syndrome, PURPLE, for ''painful purpuric ulcers with reticular patterning on the lower extremities.'') Some patients with PURPLE may have defective release of endogenous t-PA or increased levels of t-PA inhibitor in plasma. 3 A specific fibrinolytic factor produced by endothelial cells, t-PA, given over a period of days in smaller doses than when given as a single dose for myocardial infarction, might lyse small cutaneous thrombi (which appear to be a major factor in the pathogenesis of PURPLE). Klein and Pittelkow 1 gave 10 mg of t-PA intravenously over a 4-hour period for 14 days with or without aspirin and heparin. Five of six patients were
Hyunjoo Choi - One of the best experts on this subject based on the ideXlab platform.
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Livedoid Vasculitis responding to PUVA therapy.
International journal of dermatology, 2001Co-Authors: Jeong Ho Lee, Hyunjoo Choi, Soo Min Kim, Seung‐kyung Hann, Yoon‐kee ParkAbstract:Background Livedoid Vasculitis is a chronic disorder manifested as recurrent, painful, reticulated, and ulcerative lesions of the legs, which result in ivory atrophic scars with peripheral telangiectasia and hyperpigmentation. Its etiology remains obscure and therapy is difficult. In this study, we evaluated the clinical efficacy of psoralen plus UVA (PUVA) therapy and its side-effects in the treatment of Livedoid Vasculitis. Methods Eight South Korean patients with Livedoid Vasculitis were treated with UVA and 8-methoxypsoralen (8-MOP). Systemic PUVA was started with 4 J/cm2 of UVA two or three times a week, and then the dose was increased by 0.5 or 1 J/cm2 increments at each subsequent treatment as tolerated. The effects of treatment were evaluated using photographs of before, during, and after the study. Results All patients experienced rapid cessation of new lesion formation, significant symptom relief, and complete healing of primary lesions. The mean times for each of the above were 3.6, 5.9, and 10 weeks, and the mean cumulative doses of UVA for each of the above were 55.9, 96.8, and 197.9 J/cm2, respectively. The patients tolerated PUVA therapy well without unacceptable side-effects. Conclusions We propose that systemic PUVA using 8-MOP should be investigated further as an alternative treatment for patients with Livedoid Vasculitis.
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Livedo reticularis and Livedoid Vasculitis responding to PUVA therapy.
Journal of the American Academy of Dermatology, 1999Co-Authors: Hyunjoo Choi, Seung-kyung HannAbstract:Livedo reticularis is a mottled blue discoloration of the skin, which occurs in a netlike pattern. Livedoid Vasculitis is a chronic disorder clinically manifested by recurrent painful ulcerations of the lower extremities and is characterized by the presence of smooth or depressed ivory-white lesions surrounded by hyperpigmentation and telangiectasia. We describe two patients with livedo reticularis and Livedoid Vasculitis who responded to PUVA therapy and propose that systemic PUVA with methoxsalen undergo further investigation as an alternative therapy for drug-resistant patients with livedo reticularis and Livedoid Vasculitis.