Association of IgA Vasculitis with Severe Idiopathic Thrombocytopenic Purpura: A Pediatric Case Report

Background: Various physio-pathological mechanisms can lead to cutaneous purpura, two of them are thrombocytopenia and vasculitis.

Objective: We report the case of a child developing both vasculitic and thrombocytopenic purpura.

Case report: A non-consanguineous twelve-year-old girl, with no medical history, presented to the emergency department for cutaneous rush evolving for three days. She was treated ten days ago by oral antibiotics (amoxicillin 50 mg/kg/day) for pharyngitis. As throat swab was not performed at the time, the responsible infectious agent was unknown. She was doing well after that, till one week later, when she presented with acute onset of abdominal pain and arthritis of the two knees, followed three days later by a palpable purpuric rush of the two legs. First assessment found an apyretic and hemodynamically stable child, with no externalized bleeding. Physical examination was unremarkable. The child was given ibuprofen and phloroglucinol orally. Skin biopsy showed a leukocytoclastic vasculitis with IgA deposits. Abdominal sonograph found no intussusception. Meanwhile, laboratory assessment showed inflammation (ferritin=278 ng/ml, CRP=57 mg/l, ESR=20 mm), and high plasma IgA levels. CBC showed leukocytosis=30080/mm3, granulocytosis=26140/mm3, normocytic anemia=10.9 g/dl, and thrombocytopenia=33000/mm3. After what, a myelogram was performed and proved normal. Renal involvement was in the form of mild proteinuria (660 mg/24h) without AKI. Also, ASLO levels, autoimmune disease screening, and SARS Cov-2 PCR were negative. Management consisted of 1 g/1.73m2/day of methylprednisolone for three consecutive days. By the third day of treatment, evolution was marked by the appearance of oral hemorrhagic blisters, and the aggravation of thrombocytopenia (12000/mm3). That led us to start intravenous immunoglobulins at 1 g/kg/day for two days, as well as platelets transfusion. Unfortunately, we lost the child the next day by cerebral hemorrhage.

Conclusion: he association between Henoch-Schönlein and idiopathic thrombocytopenic purpura can be very dreadful, especially in the pediatric population.