Livedoid vasculopathy
| Livedoid vasculopathy | |
|---|---|
| Other names | Livedoid vasculitis, Livedo reticularis with summer/winter ulceration , Segmental hyalinizing vasculitis. peripheral vascular disease, and vasculitis.[2] |
| Treatment | Pain management, wound care, smoking cessation, compression, antiplatelet agent, and anticoagulants.[2] |
| Frequency | 1 in 1,00,000 per year.[3] |
Livedoid vasculopathy (LV) is an uncommon thrombotic dermal
Livedoid vasculopathy has been linked to various conditions that can induce hypercoagulability, including neoplasms, autoimmune connective-tissue diseases, and inherited and acquired thrombophilias.[5]
The history, clinical findings, and histopathological analysis are combined to make the diagnosis.[5]
Prompt and suitable intervention mitigates discomfort and averts the formation of wounds and additional complications. In addition to general supportive measures, anticoagulants and antiplatelet medications can be considered the first-line treatments.[6]
Signs and symptoms
Recurrent focal non-inflammatory
Complications
Due to the tissue exposure, the primary acute complications are pain and secondary infection.
Causes
Few things are known about the origins of dermal vessel thrombosis in livedoid vasculopathy, what sets off its initial episodes and relapses, and why it primarily affects the lower limbs.[4]
Risk factors
Livedoid vasculopathy has been linked to
Patients with hematological or solid organ cancers may also experience livedoid vasculopathy. Livedoid vasculopathy may deteriorate during pregnancy, particularly in the third trimester, though fetal compromise has not been documented. Still, a sizable fraction of cases are idiopathic.[9]
Genetics
Livedoid vasculopathy is caused by a number of inherited and acquired coagulation abnormalities, such as
Mechanism
At this point, the pathomechanism of livedoid vasculopathy is not fully understood.
Diagnosis
To diagnose livedoid vasculopathy and its causes, a thorough history, dermatological examination, and laboratory work-up are necessary.[9] A skin biopsy should be performed to confirm the diagnosis of livedoid vasculopathy when it is clinically suspected. The most suitable type of biopsy is a fusiform incisional biopsy that contains subcutaneous fat.[6]
Following histopathological confirmation of the diagnosis, a more thorough evaluation of any potential underlying illnesses may be conducted. Naturally, the first steps in the assessment process should be a thorough history, a review of the systems, and a physical examination to look for any signs of underlying diseases. For every patient, thrombophilia laboratory testing is advised. More testing is necessary to determine whether thrombophilia is inherited or acquired, including looking into coagulating factors and their mutations.[6]
In the event that pertinent data suggestive of
The initial step in the clinical work-up should be considering the differential diagnoses of additional common causes of atrophie blanche.
Treatment
Although there are numerous distinct treatment modalities for livedoid vasculopathy, no published, standardized, evidence-based therapeutic strategies exist.[5] The improvement of skin lesions, avoidance of relapses, and pain relief are the main goals of treatment for livedoid vasculopathy.[15] Since not every patient responds to a single therapy approach equally, it is necessary to evaluate or combine a number of treatment options.[6]
Treating pain related to ulcers with
The most widely documented treatment for livedoid vasculopathy is oral anticoagulation, which directly addresses dermal vessel thrombosis.[15] The most widely used of these is rivaroxaban, which showed a significant reduction in pain after 12 weeks of therapy in an uncontrolled phase 2a trial.[18] Common substitutes are antiplatelet agents like aspirin and pentoxifylline.[4] Patients who are not responding to traditional therapies may benefit from the use of low-dose systemic thrombolytics.[19]
Epidemiology
According to estimates, the annual incidence of livedoid vasculopathy is 1:100,000, with women being affected at a ratio of 3:1. Patients may experience functional impairment for decades as the mean age of onset is in the 30s.[4]
See also
References
- ^ "Monarch Initiative". Monarch Initiative. Retrieved January 29, 2024.
- ^ a b c d e "UpToDate". UpToDate. Retrieved January 29, 2024.
- PMID 32644463. Retrieved January 29, 2024.
- ^ PMID 36285834.
- ^ PMID 36262273.
- ^ PMID 35024414.
- ^ ISSN 0365-0596.
- PMID 23348110.
- ^ PMID 27297279.
- PMID 9554296.
- ^ PMID 23582572.
- ^ S2CID 248194522.
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- ^ S2CID 25609541.
- ^ PMID 24028907.
- S2CID 25099027.
- PMID 26853646.
- PMID 1434852.
Further reading
- Criado, Paulo Ricardo; Pagliari, Carla; Morita, Thâmara Cristiane Alves Batista; Marques, Gabriela Franco; Pincelli, Thais Prota Hussein; Valente, Neusa Yuriko Sakai; Garcia, Maria Salomé Cajas; Carvalho, Jozélio Freire; Abdalla, Beatrice Martinez Zugaib; Sotto, Mirian Nacagami (2021). "Livedoid vasculopathy in 75 Brazilian patients in a single-center institution: Clinical, histopathological and therapy evaluation". Dermatologic Therapy. 34 (2): e14810. PMID 33496999.
- Hairston, Bethany R.; Davis, Mark D. P.; Pittelkow, Mark R.; Ahmed, Iftikhar (November 1, 2006). "Livedoid Vasculopathy". Archives of Dermatology. 142 (11). American Medical Association (AMA). PMID 17116831.