Immunoglobulin A (IgA) vasculitis is the most frequent form of systemic vasculitis in childhood. It usually manifests as non-thrombocytopenic purpura, petechiae, or ecchymosis at the buttocks and the lower extremities together with musculoskeletal (arthralgia or arthritis), gastrointestinal tract, and/or renal involvement.
Skin manifestations of IgA vasculitis
The rash is a characteristic sign of the disease. Typically, children with IgA vasculitis exhibit purpuric or petechial rash, especially involving the legs, including the buttocks.
However, in approximately 3% of children, more severe skin involvement can occur, marked by the development of hemorrhagic vesicles and bullae that can progress to necrosis.
In instances where skin changes deviate from the typical distribution or when the rash is particularly severe, a skin biopsy is recommended. This biopsy should include specific staining for IgA, which is a crucial step to exclude other forms of vasculitis. Notably, the absence of IgA staining in the biopsy specimens doesn’t rule out the possibility of IgA vasculitis.
The multifaceted nature of the disease emphasizes the importance of a comprehensive diagnostic approach when assessing skin manifestations in IgA vasculitis.
Association of skin involvement and disease severity in children with IgA vasculitis
Recent findings from an international multicenter study showed an association between the severity and duration of skin manifestations in IgA vasculitis and an increased risk of developing IgA vasculitis nephritis.
Certain factors, such as the presence of ulcerations and necrosis, persistent purpura lasting one month or more, and older age, have been identified as significant predictors of IgA vasculitis nephritis. Additionally, persistent purpura, male gender, and older age serve as predictors for persistent IgA vasculitis nephritis, specifically when hematuria and/or proteinuria persist for three months or longer.
The results of this study suggest the importance of careful monitoring of patients with severe and prolonged skin manifestations of IgA vasculitis. Therefore, the risk of worsened prognosis necessitates more aggressive treatment.
Semin Arthritis Rheum. 2023;61:152209
Treatment approaches based on skin manifestations in IgA vasculitis
Determining the optimal treatment approach for the bullous and necrotic forms of IgA vasculitis remains an ongoing challenge. To date, published results have not definitively demonstrated the efficacy of systemic corticosteroids in influencing the disease course or mitigating skin sequelae associated with necrotic lesions. This underscores the need for further research and tailored therapeutic strategies for these specific manifestations of IgA vasculitis.
In managing IgA vasculitis patients, a thorough follow-up is crucial to detect potential complications. Even when initial blood pressure measurements and urinalysis, with a focus on detecting hematuria and quantifying albuminuria and/or proteinuria, appear normal, a follow-up period of at least 6-12 months is recommended.
Opening questions for future research or unmet needs
Examine the optimal approach for managing patients with severe cutaneous manifestations in IgA vasculitis.
Investigate the duration of follow-up required, and identify patients at a higher risk of complications who may need more frequent and extended monitoring.
Explore the potential of personalized medicine in the treatment of IgA vasculitis/nephritis and determine the optimal timing for its implementation.
These are the objectives of the CliPs project. By responding to our questionnaire, you actively contribute to defining word-wide applied clinical treatment strategies. These strategies have the potential to become a globally recognized standard, offering the best solutions for treatment and ensuring optimal outcomes in Kawasaki disease and IgA vasculitis.
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