Background: Skin changes, which are a mandatory classification criterion of IgA vasculitis (IgAV), are most commonly typical, but approximately 2% of children may experience the most severe changes (hemorrhagic bullae and necroses). The purpose of this research was to determine whether such changes were also associated with a more severe clinical picture and the need for more intensive therapy. Methods: The study included pediatric patients with the most severe skin manifestations as part of IgAV from 8 international tertiary university medical centers. Patients were diagnosed by EULAR/PRES/PRINTO criteria. Data were analyzed descriptively and using the Fisher test. Results: A total of 41 patients with the most severe skin changes in IgAV were included in the study, of which 26 (63.4%) were male, with a median (range) age of 8.75 (1.5-17.08) years at the disease onset. Control group consisted of 596 IgAV patients who did not develop bullae and/or necroses. In most patients, IgAV started with skin manifestations (62.5%). The time from the onset of the first symptom to the first bullous/necrotic change was 4.5 (0-180) days, and from the onset of the first purpuric change to the first bullous/necrotic change 3.5 (0-180) days. Total duration of bullous/necrotic changes was 10 (4-780) days. The most prominent trigger of IgAV were infections, present in 57.5% patients. The distribution of bullae and necroses followed the distribution of the purpuric changes, predominantly affecting the lower extremities in 70% of the patients. Sequelae of the skin changes were not present in 75% of the patients, while the rest had scars and pigmentation changes. 40% of these patients developed nephritis, and the most common finding was a combination of hematuria and proteinuria. The patients with severe cutaneous manifestations were older in comparison to other patients with IgAV (mean age 8.75 years vs. 7.17 years, p = 0.008) and had statistically more frequently affected kidneys (39% vs. 20.6%, p = 0.01), however, no one developed chronic renal disease. All these patients were treated: 75% of them received methylprednisolone with a median (range) cumulative dose of 33 (4-170.46) mg/kg for a median (range) of 17 (3-298) days, while 40% were treated with nonsteroidal anti-inflammatory drugs for 5 (2-30) days. Other drugs were administered sporadically. They were significantly more frequently treated with systemic glucocorticoids (90.2% vs. 37.2%, p < 0.001). Conclusions: Patients with IgAV and the most severe cutaneous manifestations were statistically significantly more likely to develop nephritis and were treated with systemic glucocorticoids. Disclosures: None.