Association between cutaneous manifestations and clinical features of IgA vasculitis

Martina Held, Mario Šestan, Danica Grgurić, Nastasia Kifer, Ante Vidović, Marijan Frković, Marija Jelušić

Introduction: IgA vasculitis (IgAV ) is the most common systemic vasculitis in childhood. Purpuric rash is a mandatory 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.

Uvod: IgA vaskulitis (IgAV ) najčešći je sistemski vaskulitis dječje dobi. Purpurični osip ključan je kriterij za dijagnozu IgAV -a, a najčešće je rasprostranjen po donjim udovima i gluteusima, iako može biti proširen i na atipičnim mjestima poput lica, trupa i gornjih udova. U najtežim slučajevima mogu biti prisutne ulceracije, nekroze i bule. Cilj: Utvrditi osobitosti kožnih promjena u bolesnika s IgAV -om te ispitati njihovu povezanost s kliničkim značajkama. Ispitanici i metode: Retrospektivna analiza podataka bolesnika s IgAV -om dijagnosticiranih i liječenih u Referentnom centru za pedijatrijsku i adolescentnu reumatologiju Ministarstva zdravstva RH, u razdoblju od siječnja 2009. do prosinca 2021. godine. Rezultati: IgAV je dijagnosticiran u 234 bolesnika, 124 dječaka i 110 djevojčica s medijanom (rasponom) dobi u trenutku dijagnoze 6,5 (4,5 – 8,2) godina. Svi su bolesnici imali kožni osip, a u njih 127 (54,3%) IgAV je i započeo osipom. Kožne promjene najčešće su bile zastupljene u obliku palpabilne purpure i/ili petehija (87,2%) i u svih bolesnika bile su lokalizirane po donjim udovima. U 103 bolesnika (44%) kožni osip se dalje proširio na ruke, trup i/ili lice. U 47 bolesnika (20,1%) došlo je do barem jednog recidiva kožnih promjena. Najteže kožne promjene u vidu ulceracija i nekroza razvilo je 11 bolesnika (4,7%). Bolesnici s kožnim promjenama proširenim iznad donjih udova imali su statistički značajno češće zahvaćen gastrointestinalni sustav u odnosu na bolesnike s kožnim osipom ograničenim na donje udove i glutealno (50,5% u odnosu na 36,6%, p=0,033), veću pojavnost nefritisa (IgAVN ) (31,1% u odnosu na 19,8%, p=0,048) te su češće liječeni sistemskim glukokortikoidima (68% u odnosu na 52,7%, p=0,018) i inhibitorima angiotenzin konvertaze (14,5% u odnosu na 5,3%, p=0,016). Gotovi svi bolesnici s ulceracijama i nekrozama zahtjevali su liječenje sistemskim glukokortikoidima u odnosu na sve preostale bolesnike (90,9% u odnosu na 57,8%, p=0,031). Zaključak: Uočili smo da bolesnici s kožnim osipom proširenim iznad donjih udova imaju češće zahvaćen gastrointestinalni sustav i češću pojavu IgAVN -a. Učestalost ulceracija i nekroza u IgAV -u rjeđa je od klasične slike kožnog osipa i takvi bolesnici zahtijevali su liječenje sistemskim glukokortikoidim.