P2.K217: Bullous IgA Vasculitis: A Unique Presentation and Potential Marker of Severity

Introduction: Immunoglobulin A (IgA) vasculitis, also known as Henoch–Schönlein purpura, is the most common childhood vasculitis. Palpable purpura is one of the pathognomonic features of IgA vasculitis, with over 70% of patients experiencing this symptom at onset. However, rarely in children ( < 2% of cases), patients may present with hemorrhagic bullous lesions or have evolution of purpura into bullae. We present a case of IgA vasculitis and nephritis presenting with bullous lesions and severe nephrotic syndrome.

Case Description: A 5-year-old female presented with one week of worsening rash of the lower extremities. It began as a flat and maculopapular rash, initially diagnosed as IgA vasculitis. Since diagnosis, the rash had progressed to include pustules, erosions, and crusting, with extension to the upper extremities, abdomen, neck, face, and ears. At presentation she was found to have edema of the hands, feet, and periorbital area, along with hematuria, proteinuria, hypoalbuminemia and hypertension. She was started on lisinopril with as needed isradipine for hypertension.
On day one of admission, she developed severe periumbilical pain, without evidence of intussusception on ultrasound, and was started on methylprednisolone. She had no improvement in her rash by day two and there was concern that she may have an infectious or rheumatologic process, therefore punch biopsy was performed. The lesions were shown to be consistent with IgA vasculitis.
Her rash began improving on day four and she was discharged home on lisinopril, furosemide, and prednisolone. After discharge, she was additionally started on amlodipine. Following four weeks on high-dose steroids, she continued with nephrotic range proteinuria and was transitioned to mycophenolate while steroids were weaned. At eight weeks post-discharge, mycophenolate was increased due to persistent proteinuria. At twelve-weeks post-discharge, she continues with proteinuria on mycophenolate and steroid wean.

Discussion: Many of the patients with bullous IgA vasculitis will undergo skin biopsy, which may be unnecessary and increase the risk of complications. These lesions can take several weeks to fully heal, so time itself is likely not a strong indication to perform biopsy. Regarding severity of disease, there is limited but conflicting literature on the correlation between skin severity and kidney involvement. This patient presented with both severe skin lesions with a unique morphology and severe nephrotic syndrome resistant to steroids, an unusual course for IgA vasculitis.

Conclusion: By being aware of this unique presentation of bullous IgA vasculitis, unnecessary skin biopsies can be avoided in patients who present with skin purpura consistent with IgA vasculitis that advances into bullae. Current evidence is nonconclusive regarding the link between severity of skin findings and renal involvement, however this unique case suggests these findings may be a useful predictor which can broaden our understanding with research into the severity of disease and skin findings on clinical presentation.