Livedoid Vasculopathy

A rare cutaneous thrombotic disease due to occlusion of dermal vessels. It is characterized by purpuric maculae and ulcerations especially during summer which form scars called atrophie blanche. It is more associated with other syndromes (e.g., PROTEIN C DEFICIENCY; HYPERHOMOCYSTEINEMIA). Livedo reticularis with systemic involvement and stroke is SNEDDON SYNDROME.

Also Known As:

Atrophic Blanche, Idiopathic; Blanches, Idiopathic Atrophic; Livedo Vasculitides; Livedoid Vasculopathies; Vasculitis, Livedo; Vasculitis, Livedoid; Vasculopathy, Livedoid; Idiopathic Atrophic Blanche; Livedo Reticularis with Summer Ulceration; Livedo Vasculitis; Livedoid Vasculitis; White Atrophy

Networked: 177 relevant articles (22 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pain (Aches)
2.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
3.	Varicose Ulcer (Ulcer, Venous)
4.	Sickle Cell Trait
5.	Lipodermatosclerosis

Experts

1.	Criado, Paulo Ricardo: 7 articles (01/2021 - 12/2013)
2.	Gao, Yimeng: 5 articles (08/2022 - 12/2020)
3.	Jin, Hongzhong: 5 articles (08/2022 - 12/2020)
4.	Goerge, Tobias: 5 articles (07/2018 - 08/2010)
5.	Kirsner, Robert S: 5 articles (12/2017 - 12/2013)
6.	Alavi, Afsaneh: 4 articles (07/2015 - 12/2013)
7.	Criado, P R: 3 articles (11/2015 - 11/2010)
8.	Strölin, Anke: 2 articles (09/2021 - 02/2016)
9.	Cho, Soyun: 2 articles (01/2021 - 09/2015)
10.	Pincelli, Thais Prota Hussein: 2 articles (01/2021 - 01/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Livedoid Vasculopathy:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 09/01/2003 - "Intravenous immunoglobulin in livedo vasculitis: a new treatment option?" 10/01/2014 - "Efficacy of intravenous immunoglobulins in livedoid vasculopathy: long-term follow-up of 11 patients." 01/01/2022 - "Intravenous immunoglobulin (IVIG) was reported to be the third most used monotherapy in livedoid vasculopathy (LV). " 01/01/2022 - "Efficacy and safety of intravenous immunoglobulin for treating refractory livedoid vasculopathy: a systematic review." 01/01/2020 - "Intravenous immunoglobulin in resistant livedoid vasculopathy: Analysis of a case series."
2.	RivaroxabanIBA 02/01/2016 - "We investigated whether rivaroxaban is effective for the treatment of livedoid vasculopathy. " 08/01/2022 - "Comparative Efficacy of Rivaroxaban and Immunoglobulin Therapy in the Treatment of Livedoid Vasculopathy: A Systematic Review." 02/01/2016 - "Anticoagulation with rivaroxaban for livedoid vasculopathy (RILIVA): a multicentre, single-arm, open-label, phase 2a, proof-of-concept trial." 01/01/2023 - "Excellent Long-term Clinical Response to Rivaroxaban in Livedoid Vasculopathy: A Retrospective Study." 01/01/2021 - "We conducted a systematic review of the efficacy and safety of rivaroxaban for treatment of livedoid vasculopathy (LV) by searching the PubMed, Cochrane and Embase databases. "
3.	AnticoagulantsIBA 07/24/2017 - "Efficacy of new low-dose oral anticoagulants in recalcitrant livedoid vasculopathy." 06/01/2011 - "Livedoid vasculopathy: fast involution after anticoagulant and hyperbaric oxygen therapy." 01/01/2023 - "Early initiation of treatment with antiplatelets and anticoagulants is recommended to prevent the progression of livedoid vasculopathy (LV).Anti-TNF agents can be tried in refractory LV for rapid relief of pain.Intravenous" 12/10/2014 - "Livedoid vasculopathy is associated with increased thrombophilia in the cutaneous microcirculation and the continuous use of anticoagulants helps improve the symptoms. " 11/01/2010 - "Frequency of thrombophilia determinant factors in patients with livedoid vasculopathy and treatment with anticoagulant drugs--a prospective study."
4.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 06/01/2012 - "Livedoid vasculopathy associated with sickle cell trait: significant improvement on aspirin treatment." 11/01/2003 - "In this case series, the clinical profile of 6 patients with livedoid vasculitis and their treatment response to aspirin and pentoxifylline are reported. " 10/01/2015 - "Successful Treatment of Livedoid Vasculitis with Primary Antiphospholipid Syndrome by Using Aspirin and Low Dose Warfarin Combination Therapy." 01/01/1988 - "Two patients with livedo vasculitis were treated successfully with antiplatelet drugs including ticlopidine hydrochloride, dipyridamole, and low-dose aspirin. " 04/01/2004 - "Dermatohistologic findings and laboratory tests of the patient's coagulation system revealed the diagnosis of livedoid vasculopathy with heterozygous factor V Leiden mutation and sticky platelet syndrome type II. Systemic treatment with acetylsalicylic acid and heparin as well as topical therapy with disinfectant and granulation-inducing agents resulted in improvement of the skin lesions."
5.	Danazol (Azol)FDA LinkGeneric 01/01/1997 - "We concluded that low-dose danazol was effective in the treatment of livedoid vasculitis, without unacceptable side effects." 01/01/1997 - "Our purpose was to test the efficacy of low-dose danazol in the treatment of livedoid vasculitis. " 01/01/1997 - "Seven patients with active lesions of livedoid vasculitis were treated with low-dose danazol (200 mg, orally, daily). " 01/01/1997 - "Low-dose danazol in the treatment of livedoid vasculitis." 11/01/1998 - "Livedoid vasculitis with anticardiolipin antibodies: improvement with danazol."
6.	baricitinibIBA 01/01/2022 - "Improvement in livedoid vasculopathy was observed with significant regression in the clinical scores after baricitinib treatment. " 01/01/2022 - "Baricitinib is potentially effective in the treatment of refractory livedoid vasculopathy." 01/01/2022 - "Our study showed that baricitinib treatment was safe and could significantly relieve the signs and symptoms of livedoid vasculopathy. " 11/01/2023 - "Refractory livedoid vasculopathy successfully treated with baricitinib." 01/01/2023 - "Refractory livedoid vasculopathy successfully treated with baricitinib."
7.	Warfarin (Coumadin)FDA LinkGeneric 09/01/2017 - "We hereby present our experience of low-dose oral warfarin in the management of three cases of livedoid vasculopathy." 09/01/2017 - "Use of Low-dose Oral Warfarin in Three Cases of Livedoid Vasculopathy." 11/01/2011 - "Livedoid vasculopathy; favorable clinical response with low dose warfarin." 03/01/2008 - "Ulcerations caused by livedoid vasculopathy associated with a prothrombotic state: Response to warfarin." 01/01/2010 - "This case suggests that administration of low-dose warfarin is an effective therapy of choice for patients with livedoid vasculopathy."
8.	Anticardiolipin AntibodiesIBA 01/01/2011 - "The significance of anticardiolipin antibody and immunologic abnormality in livedoid vasculitis." 09/01/2000 - "Livedoid vasculopathy is normally described as occurring as a sole entity, however thera have been reports of an association with anticardiolipin antibodies. " 01/01/1999 - "Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. " 09/01/2000 - "[Livedoid vasculopathy (white atrophy) associated with anticardiolipin antibodies]." 03/01/1989 - "She also had painful scarring ulcers of the lower legs resembling livedo vasculitis and a circulating anticardiolipin antibody. "
9.	Plasminogen Activators (Plasminogen Activator)IBA 07/01/2011 - "Enhanced functional stability of plasminogen activator inhibitor-1 in patients with livedoid vasculopathy." 11/01/2006 - "Livedoid vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity (4G/4G) treated successfully with tissue plasminogen activator." 10/01/2022 - "Livedoid Vasculopathy Associated With Plasminogen Activator Inhibitor-1 Polymorphisms Treated With Factor Xa Inhibitors." 06/01/2017 - "Enhanced mRNA expression of plasminogen activator inhibitor-1 in livedoid vasculopathy lesions." 10/01/2011 - "Successful long-term use of intravenous immunoglobulin to treat livedoid vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity."
10.	Oxygen (Dioxygen)IBA 03/01/2013 - "Livedoid vasculopathy successfully treated with hyperbaric oxygen." 09/01/2003 - "Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen." 07/01/1986 - "[Indirect lymphangiography and transcutaneous measurement of oxygen pressure in white atrophy]." 02/01/2006 - "To analyse the long-term effect and safety of hyperbaric oxygen (HBO) therapy in treating livedoid vasculopathy. " 11/01/1984 - "In avascular skin areas (scar tissue, white atrophy) tcPO2 was measured at 0 mm Hg. No capillaries, or a greatly reduced number, were visible at such sites, resulting in a distance between capillary and cathode tip of the oxygen sensor of greater than 100 micron. "

Therapies and Procedures

1.	Therapeutics 01/01/1996 - "We propose that PGI2 analogue therapy is useful for the treatment of livedo vasculitis, since the drug upregulates TM expression in this disorder." 10/01/2014 - "Evidence for the efficacy of various therapies of livedoid vasculopathy (LV) is limited. " 09/01/2003 - "To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. " 02/01/2022 - "Livedoid vasculopathy is a recurrent thrombo-occlusive vasculopathy of cutaneous blood vessels and its standard or first-line therapy is still controversial. " 01/01/2022 - "This article presents the current state of knowledge about livedoid vasculopathy and proposes an algorithmic approach for diagnosis and therapy."
2.	Plant-Based Diet 02/23/2021 - "Remission of long-standing livedoid vasculopathy using a whole foods plant-based diet with symptoms recurrent on re-challenge with standard Western diet."
3.	PUVA Therapy 02/01/2001 - "Livedoid vasculitis responding to PUVA therapy." 02/01/1999 - "Livedo reticularis and livedoid vasculitis responding to PUVA therapy." 02/01/2001 - "In this study, we evaluated the clinical efficacy of psoralen plus UVA (PUVA) therapy and its side-effects in the treatment of livedoid vasculitis. " 02/01/1999 - "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."
4.	Hyperbaric Oxygenation 01/01/2022 - "Medium-pressure hyperbaric oxygen therapy for livedoid vasculopathy." 09/01/2015 - "This case is being presented for the novel option of hyperbaric oxygen therapy in livedoid vasculopathy, which by itself is rarely reported in this part of the world. " 04/01/2013 - "Livedoid vasculopathy managed with Hyperbaric oxygen therapy." 10/01/2012 - "Livedoid vasculopathy managed with hyperbaric oxygen therapy." 06/01/2011 - "We concluded that anticoagulation associated with hyperbaric oxygenation may be benefit for the treatment of patients with livedoid vasculopathy. "
5.	Pain Management 01/01/2023 - "Pain Management Options in a Patient with Livedoid Vasculopathy and Peripheral Neuropathy." 01/01/2022 - "Real-world data on pain management and effectiveness of anti-tumour necrosis factor agents in refractory livedoid vasculopathy."