Livedo Vasculopathy vs Small Vessel Cutaneous Vasculitis

Abstract

To assess the role of platelets and lymphocyterelated immunological mechanisms in livedo vasculopathy (LV) and cutaneous small vessel vasculitis (CSVV). Livedo vasculopathy is thought to be related to the thrombotic occlusion of small and medium-sized dermal vessels. Cutaneous small vessel vasculitis comprises a heterogeneous group of disorders in which the main pathogenetic events could be modulated by circulating cytokines.Design: Case series study of 2 groups of patients affected respectively with LV and CSVV. Setting: A large clinical and research institute for the study and treatment of cutaneous diseases. Patients: Consecutive patients with clinically and histologically proved idiopathic LV (n = 8) and CSVV (n = 20) were studied and compared with healthy donors (n = 20). Patients with potentially correlated systemic diseases were excluded. Main Outcome Measures: Surface expression of platelet P-selectin and circulating level of interleukin (IL) 1␤, tumor necrosis factor ␣ (TNF-␣), IL-8, IL-2, and soluble IL-2 receptor.Results: The IL-2 and soluble IL-2 receptor levels were significantly higher in serum samples from patients with

Livedovaskulopathie – Aktuelles zur Diagnostik und Therapie des Hautinfarkts

“…Nach aktuellem Kenntnisstand ist die Livedovaskulopathie charakterisiert durch eine Okklusionserkrankung der kapillären Mikrozirkulation, die zur kutanen Ischämie und Infarzierung führt [9][10][11]. Häufi g lassen sich bei den betroffenen Patienten abnorme Gerinnungsparameter und weitere Livedovaskulopathie-assoziierte Faktoren detektieren, die eine übermäßig anhaltende Thrombosierung der Mikrozirkulation bedingen (Tabelle 1).…”

Systemic lupus erythematosus with refractory ulcerated livedoid vasculopathy: Successful treatment with intravenous immunoglobulin and warfarin

“…Livedoid vasculopathy (LV) is characterized by livedo reticularis and recurrent painful ulcerations 1. Histologically, LV shows thrombus formation and fibrin occlusion, involving dermal vessels, suggesting that the pathogenesis of LV is hypercoagulability 2.…”

Vasculopatia livedoide: uma doença cutânea intrigante

“…This condition has extensive synonymy: livedo vasculitis, segmental hyalinizing vasculitis, livedoid vasculitis, livedo reticularis with summer ulcerations, livedo reticularis with winter ulcerations, Milian's white atrophy (atrophie blanche) and PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities). [1][2][3][4] From a clinical point of view, it is characterized by lesions that begin as painful punctate or lenticular purpuric macules and/or papules on the lower limbs, especially on the ankles and dorsum of the feet. These lesions usually undergo ulceration.…”

“…Then, they heal slowly, 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 in the lower third of the limbs. 5 The estimated prevalence of LV in the U.S. general population is around 1 case per 100,000 people per year.…”

“…[6][7][8][9] LV was originally described as a clinical manifestation of vasculitis. However, at present, the main physiopathogenic mechanism considered is a vasoocclusive phenomenon due to intraluminal thrombosis of dermal venules 1 . Papi et al substantiated this concept based on the clinical evolution of the disease, its improvement under antiplatelet and fibrinolytic therapy and on the detection of high levels of fibrinopeptide A.…”