Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement

Abstract

In this study, outcomes of patients with leukocytoclastic vasculitis (LCV) were analyzed focusing on clinical, histopathology and laboratory findings, relapses, and survival.Data from patients with cutaneous vasculitis diagnosed between January 1, 2000, and December 31, 2010, at Dijon University Hospital (France) were retrospectively reviewed. LCV was defined as perivascular neutrophilic infiltrate, endothelial cell nuclear swelling, extravasation of red blood cells, and/or fibrin deposition in vessels. Patients were classified according to the 2012 Chapel Hill Consensus Conference. Relapses were defined as the recurrence of vasculitis symptoms after a period of remission >1 month. Time to relapse and/or death was calculated from the date of diagnosis. Univariate and multivariate (Cox model) analyses were performed.A total of 112 patients (57 males and 55 females), with a mean age of 60 ± 19 (18–98) years, were analyzed. Overall follow-up was 61 ± 38 months. At diagnosis, all patients had skin lesions, purpura being the most common (n = 83). Lesions were associated with systemic involvement in 55 (51%) patients. Only 41 (36.6%) patients received specific treatment: glucocorticoids in 29 of 41 (70.7%) and immunosuppressants in 9 of 41 (22%). Sixty-two patients (55%) had LCV due to underlying causes, 29 (25.9%) had single-organ cutaneous small vessel vasculitis (SoCSVV), and 21 (18.8%) had unclassifiable LCV. Twenty patients of the cohort (18%) experienced relapse, 14 ± 13 (1–40) months after the diagnosis of LCV. None of the 29 patients with SoCSVV relapsed. Independent risk factors for relapse were vascular thrombosis in the biopsy [hazard ratio (HR) = 4.9; P = 0.017], peripheral neuropathy (HR = 9.8; P = 0.001), hepatitis (HR = 3.1; P = 0.004), and positive antineutrophil cytoplasm antibodies (ANCA, HR = 5.9 P = 0.005). In contrast, SoCSVV was a protective factor for relapse (HR = 0.12; P = 0.043).The 1-, 3-, and 6-year overall survival rates were 99%, 83%, and 71%, respectively, with no difference between relapsers and nonrelapsers (P = 0.960) or between SoCSVV and unclassifiable LCV (P = 0.588).This study demonstrates that global survival for LCV patients is good but relapses remain frequent, especially when the cutaneous biopsy shows vascular thrombosis, or in patients with peripheral neuropathy or hepatitis. Conversely, SoCSVV is a protective factor for relapse.

A Study of Aetiological Factors in the Occurrence of Cutaneous Vasculitis in a Tertiary Care Centre in North Kerala

“…9,10 A causal agent or an underlying condition has been reported in 20-85 percent of the cases. 11,12,10,13 Similar to our findings, a previous study from India had found that drugs were the most common aetiological factor in vasculitis. 14 In contrast to our study, some previous studies had shown that antibiotics rather than NSAIDs were responsible for drug-induced vasculitis.…”

Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV)

“…Recently, it has been shown that drugs and mild infections such as upper respiratory tract infections are responsible for 25% and 10% of SoCSVV, respectively. [3,4] In our study these frequencies were a little different with more infections (33.3%) than drugs (16.7%) as causal agents of SoCSVV.…”

“…However, other skin lesions, such as red macules, urticaria vasculitis, and ulcers, may be observed. [3,4] In our series, red macules were identified as the second most common clinical finding in patients with SoCSVV. Interestingly, in a multivariate logistic regression analysis, we identified macules as an independent factor of ulcers’ occurrence in the course of vasculitis.…”

“…However, the authors emphasized that the relapse rate seems to be significant, sometimes causing problems in everyday clinical practice. [3,4] Moreover, some patients with palpable purpura may later develop ulcers that significantly lengthen the duration of treatment and may be further complicated by infections or scarring.…”

Gastrointestinal Biopsy in Henoch-Schönlein Purpura: A Great Diagnostic Contribution

“…In adults, the diagnosis of HSP is more difficult, especially in atypical forms. Many other diagnoses involving LCV must be ruled out, including infection, drug effects, malignancy and connective tissue disease [5] . According to the EULAR recommendations [3] , in the case of atypical forms (atypical cutaneous manifestations or incomplete forms of disease), the presence of IgA deposits (which has a sensitivity and specificity of >80%) is required.…”