Presentation
However, at present, the main physiopathogenic mechanism considered is a vasoocclusive phenomenon due to intraluminal thrombosis of dermal venules1. [scielo.br]
On December 29, 2017, fourteen days after starting the treatment described above, the patient presented total resolution of pain, regression of hyperemia of ulcers, and signs of healing. [ncbi.nlm.nih.gov]
[…] and spots that progress to small, tender, irregular ulcers (30% of cases) Painless, irregular atrophie blanche (porcelain-white, stellate scars with red dots due to prominent capillaries) Livedo reticularis, Raynaud phenomenon and acrocyanosis may be present [dermnetnz.org]
An interesting case of livedoid vasculopathy occurred in a child in an area on the leg where a cutaneous hemangioma had been present. [emedicine.medscape.com]
Entire Body System
- Swelling
[…] over weeks or months, resulting in porcelainwhite atrophic scars (white atrophy), punctate telangiectasia, and livedoid brownish pigmentation, usually accompanied by livedo racemosa. 1 The disease usually affects the legs bilaterally, often causing swelling [scielo.br]
Cardiovascular
- Thrombosis
Keywords: Leg ulcer; Livedo reticularis; Thrombophilia; Thrombosis; Venous thrombosis INTRODUCTION Livedoid vasculopathy (LV) is a dermatosis whose management can be challenging. [scielo.br]
Background Livedoid vasculopathy (LV), or livedoid vasculitis, is a hyalinizing vascular disease characterized by thrombosis and ulceration of the lower extremities. [emedicine.medscape.com]
Livedoid vasculopathy and recurrent thrombosis in a patient with lupus: seronegative antiphospholipid syndrome? Sopeña B, Pérez-Rodríguez MT, Rivera A, Ortiz-Rey JA, Lamas J, Freire-Dapena MC. Sopeña B, et al. [pubmed.ncbi.nlm.nih.gov]
Histopathology reveals hyalinisation, thickened blood vessel walls, fibrin deposition, vascular occlusion by thrombosis and minimal inflammation. [dermnetnz.org]
During the pregnancy, there was no edema in the left lower limb, venous dilation, varicose veins, dermatosclerosis, phlebitis, deep venous thrombosis, or other signs (Figure 4). [ncbi.nlm.nih.gov]
- Acrocyanosis
[…] red or purple marks and spots that progress to small, tender, irregular ulcers (30% of cases) Painless, irregular atrophie blanche (porcelain-white, stellate scars with red dots due to prominent capillaries) Livedo reticularis, Raynaud phenomenon and acrocyanosis [dermnetnz.org]
Musculoskeletal
- Osteoporosis
43 However, patients should be informed of the possibility of bleeding events while undergoing anticoagulant therapy, as well as contraindications to anticoagulation must be strictly observed prior to initiating treatment. 43 Moreover, the risk of osteoporosis [scielo.br]
Skin
- Papule
It begins with purpuric papules or macules that develop into painful ulcers, mainly involving the ankles and feet. In this case report, we describe diagnosis and treatment in a young pregnant patient, with excellent clinical response. [pubmed.ncbi.nlm.nih.gov]
Livedoid vasculopathy is a clinical diagnosis, supported by skin biopsy of a red papule or the edge of a new ulcer. [dermnetnz.org]
A physical examination may reveal lesions in different stages of evolution, usually associated with livedo racemosa. 8 During its evolution, purpuric and erythematous papular plaques and papules are formed. [scielo.br]
- Foot Ulcer
Venous insufficiency with venous eczema and stasis ulcer Arterial insufficiency, arterial ulceration and diabetic foot ulcer Rheumatoid arthritis Systemic lupus erythematosus Systemic sclerosis Small vessel vasculitis Cutaneous polyarteritis nodosa Malignant [dermnetnz.org]
- Cutaneous Manifestation
However, representation of the dermo-hypodermal junction is necessary, since there are situations in which the lesions clinically present as LV, but represent a morphological manifestation of cutaneous polyarteritis nodosa. [scielo.br]
- Dermatitis
[…] small vessel vasculitis (leukocytoclastic vasculitis) and cutaneous polyarteritis nodosa (especially when there are symptoms of mononeuritis multiplex), in addition to antiphospholipid syndrome, chronic venous stasis, pyoderma gangrenosum, factitious dermatitis [scielo.br]
Neurologic
- Hyperesthesia
Some patients experience symptoms of paresthesia or hyperesthesia, characterized as mononeuritis multiplex. 10 The involvement of the peripheral nervous system possibly occurs due to areas of multifocal ischemia, resulting from the deposition of fibrin [scielo.br]
- Confusion
[…] in an early stage of the disease. 21 It should be noted that these fibrinoid deposits (in the lumen and on walls) are significant for the diagnosis of LV and occur at all stages. 21 It is important to note that these fibrinoid deposits should not be confused [scielo.br]
Treatment
In this case report, we describe diagnosis and treatment in a young pregnant patient, with excellent clinical response. [ncbi.nlm.nih.gov]
Renal toxicity and the immunosuppressive effects of the drug do not allow for its use on an uninterrupted basis in the treatment of LV. However, it may be used as rescue treatment when other treatment forms have failed. [scielo.br]
Livedoid vasculopathy in a patient with lupus anticoagulant and MTHFR mutation: treatment with low-molecular-weight heparin. J Thromb Thrombolysis. 2012 May 17. [QxMD MEDLINE Link]. [emedicine.medscape.com]
Case Reports Livedoid Vasculopathy: diagnosis and treatment in pregnant women Alexandre Sacchetti Bezerra et al. J Vasc Bras. 2020. [pubmed.ncbi.nlm.nih.gov]
Prognosis
The average age at diagnosis is 32 years and it is most prevalent in people aged 15-50 years. [7] Prognosis Livedoid vasculopathy, although painful, is not associated with any loss of life or limb. [emedicine.medscape.com]
Etiology
Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Discipline Sarcoidosis. Granulomatosis of undetermined origin. Connective tissue diseases. [pascal-francis.inist.fr]
DISCUSSION LV is a disease with unknown etiology.2,3 In addition to vascular involvement, patients with LV also have nervous system involvement, possibly caused by deposits of fibrin and thrombin in the vasa nervorum.6 The disease may be classified as [ncbi.nlm.nih.gov]
The fibrin cuffs are more an indication of disturbed microcirculation rather than an etiologic factor in chronic venous insufficiency; therefore, this theory is of uncertain accuracy. [emedicine.medscape.com]
Regardless of the etiology involved in LV, the pathogenic changes occur in the dermis, in contrast to hypodermitis, in which the pathogenic substrate is located in the subcutaneous tissue. 21 In LV, there are no signs of true vasculitis in the form of [scielo.br]
Epidemiology
The following is a summary of the epidemiological and testing data: Approximately 71% were women Mean age of 45 years Age range of 10-85 years Bilateral lower extremity disease in 80.8% Ulceration in 68.9% Atrophie blanche in 71.1% Decreased transcutaneous [emedicine.medscape.com]
Pathophysiology
Livedoid vasculopathy and its pathophysiology are clearly distinguished from inflammatory vasculitis and thus require a different therapeutic approach. [ncbi.nlm.nih.gov]
[…] in 17.9% Anticardiolipin antibodies in 28.6% Increased homocysteine levels in 14.3% Biopsy specimens showing intraluminal thrombosis in 97.8% Biopsy specimens with direct immunofluorescence test results showing multiple vascular conjugates in 86.1% Pathophysiology [emedicine.medscape.com]
Prevention
The prevention of irreversible residual scarring and improving the quality of life of patients in this often misdiagnosed disease is one of the main treatment goals. [ncbi.nlm.nih.gov]
Various drug therapies may be prescribed to enhance blood flow and prevent blood clotting: Pentoxifylline (alters blood viscosity and red cell flexibility) Antiplatelet agents (eg, aspirin, dipyridamole) Fibrinolytic agents (eg, danazol, tissue plasminogen [dermnetnz.org]
[…] the treatment, not exceeding 1 to 2 mg daily, with daily slow increments of 1 to 2mg until the dose required to reach the desired INR is achieved, or associate it in the first 3 to 5 days with subcutaneous heparin. 91 This practice has been used to prevent [scielo.br]
The cuff establishes a barrier that prevents oxygen and nutrients from reaching the skin. However, Maessen-Visch et al [13] note that fibrin is an effective barrier to prevent the diffusion of oxygen to tissue. [emedicine.medscape.com]