Association between cutaneous manifestations and clinical features of IgA vasculitis

Abstract

Introduction: iga vasculitis (igav) is the most common systemic vasculitis in childhood. Purpuric rash is a man-datory criterion for diagnosing igav, it is mostly localized on the lower extremities and gluteal region, although it can also appear atypically affecting the face, trunk and upper extremities. in the most severe cases, ulcerations, necrosis and bullae can be present. Objectives: to evaluate the characteristics of cutaneous manifestations in patients with igav and to examine its association with clinical features. Subjects and methods: Retrospective analysis of data from patients with igav diagnosed and treated at the Referral centre for Paediatric and adolescent Rheumatology of the ministry of health of the Republic of croatia, in the period from january 2009 to December 2021. Results: igav was diagnosed in 234 patients, 124 boys and 110 girls with the median (range) age at the time of diagnosis of 6.5 (4.5–8.2) years. all patients had a purpuric rash, and in 127 of them (54.3%) igav began with a rash. cutaneous manifestations were most often presented in the form of palpable purpura and/or petechiae (87.2%) and in all patients were localized on the lower extremities. in 103 patients (44%) purpuric rash spread further to the upper extremities, trunk and/or face. at least one skin relapse occurred in 47 patients (20.1%). The most severe cutaneous manifestations which included ulcerations and necrosis developed in 11 patients (4.7%). Patients with cutaneous manifestations spread above the waist had a more statistically significant gastrointestinal involvement compared to patients with cutaneous manifestations affecting the lower extremities and gluteal region (50.5% vs. 36.6%, p=0.033), higher incidence of iga vasculitis nephritis (igavn) (31.1% vs. 19.8%, p=0.048) and were more frequently treated with systemic glucocorticoids (68% vs. 52.7%, p=0.018) and angiotensin-converting enzyme inhibitors (14.5% vs. 5.3%, p=0.016). almost all patients with ulcerations and necrosis required treatment with systemic glucocorticoids compared to the rest (90.9% vs. 57.8%,p=0.031). Conclusion: We observed that patients with purpuric rash spread above the waist have more frequently affected gastrointestinal system and a higher incidence of igavn. The prevalence of ulcerations and necrosis in igav is less common than the standard purpuric rash and this group of patients required systemic glucocorticoid therapy.