Direct immunofluorescence findings in livedoid vasculopathy: a 10‐year study and literature review

Abstract

Summary Background Direct immunofluorescence (DIF) findings in patients with livedoid vasculopathy (LV) may have benefits for disease differentiation when clinical presentations and/or histopathological findings are inconclusive. Aim To investigate DIF findings in patients with a clinical and histopathological diagnosis of LV. Methods DIF findings of 62 patients with LV were analysed, and the published literature in the PubMed database was also reviewed and summarized. Results This study demonstrated deposition of immunoreactants in blood vessels (BVs) in 59 of the 62 patients (95.2%), and almost all cases were positive for a combination of multiple immunoreactants. Complement C3 and IgM formed the most common combination. The most common pattern was deposition in BVs and at the dermoepidermal junction (DEJ) (59.3%), followed by deposition in BVs alone (40.7%). Immunoreactant deposition in BVs involved superficial BVs with or without deep BVs. The median age of patients with positive DIF findings was significantly higher than that of patients with negative DIF findings for LV (P < 0.03). More recent lesions (present for < 6 months) had a significantly higher percentage of positive results than older lesions (present for ≥ 6 months) (85.2% vs. 14.8%, respectively; P < 0.001). Conclusion In both the present study and in the published literature, DIF study in patients with LV showed positive immunoreactants ranging from 42.9% to 100%. C3 and IgM were the most common immunoreactants deposited in BVs, while the most common pattern was immunoreactant deposition in BVs and at the DEJ. Older patients and those with more recent lesions (< 6 months) had a significantly higher percentage of positive DIF results for LV than did younger patients and those with older lesions (≥ 6 months).

Livedoid vasculopathy – A diagnostic and therapeutic challenge

“…Interestingly, in LV multiple immunoreactants, especially complement factor C3 (C3), fibrinogen and Immunoglobulin M (IgM), less Immunoglobulin A (IgA) and Immunoglobulin G (IgG), can be found in direct immunofluorescence stainings, although these findings are rated as non-specific and non-diagnostic ( 8 , 15 , 40 , 41 ). The most reported pattern is granular deposition in the walls of blood vessels combined with or without depositions at the dermoepidermal junction ( 42 ). Positive results in the direct immunofluorescence were statistically significant more frequent in older patients and more recent lesions (<6 months) ( 8 ).…”

Livedoid vasculopathy: A review with focus on terminology and pathogenesis

“…Direct immunofluorescence studies have shown complement and immunoglobulin (C3>IgM>IgG>IgA) deposition in vessel walls in most patients with LV. 19,20 Immunoreactant deposition is not specific to LV, and may be found in most inflammatory disorders of the skin. 21…”

“…1,15 However, most immunohistopathologic studies of LV show complement and immunoglobin deposition in vessel walls in both primary and secondary forms of the disorder. 2,19,20 C3 is the most predominant immunoreactant, followed by IgM, IgG, and IgA. Whether the presence of lymphocytes and immunoreactants represents the primary pathologic process or is a secondary reaction to thrombosis is unclear.…”

Livedoid vasculopathy: A multidisciplinary clinical approach to diagnosis and management

“…DIF demonstrates immunoglobulin deposition (multiple immunoreactants, especially C3 and immunoglobulin [Ig] M, followed by IgA, and IgG), fibrin, and complement components, although DIF findings are thought to be nonspecific and nondiagnostic ( Criado et al, 2011 ; Khenifer et al, 2009 ; Nuttawong et al, 2021 ). In the literature, the incidence of positive DIF in patients with LV ranged from 42.9% to 100% ( Criado et al, 2014 ; Hsiao and Wu, 2010 ; Nuttawong et al, 2021 ; Schroeter et al, 1971 ; 1975 ). Older patients and more recent lesions (<6 months) had a significantly higher percentage of positive DIF results for LV ( Nuttawong et al, 2021 ).…”

“…In the literature, the incidence of positive DIF in patients with LV ranged from 42.9% to 100% ( Criado et al, 2014 ; Hsiao and Wu, 2010 ; Nuttawong et al, 2021 ; Schroeter et al, 1971 ; 1975 ). Older patients and more recent lesions (<6 months) had a significantly higher percentage of positive DIF results for LV ( Nuttawong et al, 2021 ).…”