Abstract
Background : Henoch Schonlein purpura (HSP) is an acute small vessel vasculitis. It is the most common vasculitis in children. Although the cause is unknown, IgA seems to play a central role in the pathogenesis of Henoch Schonlein purpura. The major clinical features include a palpable purpuric rash on the lower extremities, abdominal pain or renal involvement, and arthritis. Cutaneous manifestations are the essential elements in the diagnosis of Henoch Schonlein purpura. The palpable purpura is characteristically 2 to 10mm in diameter and is usually present on the lower extremities. There are no specific diagnostic tests available for diagnosing this condition. Laboratory studies are useful to exclude other conditions that may mimic Henoch Schonlein purpura. In majority of the cases, the disease is self-limited. Relapsing can occur, in particular during the first year of the disease. There is no consensus on a specific treatment. Corticosteroids are effective in rapid resolution of renal and abdominal manifestations. Immunosuppressive drugs, such as Mycophenolate Mofetil may be a better treatment choice in case of renal involvement.Case report : We report a case of a 14 years old girl affected from recurrent Henoch Schonlein Purpura. From the age of nine years patient presented three episodes of purpura with gastrointestinal involvement, in particular hematemesis, abdominal pain and diarrhoea. Each episode was treated with high doses of corticosteroids (methylprednisolone in vein or prednisone per os). Patient came to our Department during the third episode of Purpura. In consideration of the recurrence of the Henoch Schonlein Purpura and the gastrointestinal involvement we decided to start Mycophenolate Mofetil treatment. Patient’s conditions improved thanks to Mycophenolate Mofetil treatment. Conclusion: In our case of recurrent HSP Mycophenolate Mofetil treatment has been very effective, avoiding the adverse events of a prolonged steroid treatment. This experience teaches us that immunosuppressive agents may be very useful to induce and maintain remission not only in renal involvement, but in all cases of persistence, recurrence or complicated forms of Henoch Schonlein purpura in children.
Comment on: Gastrointestinal involvement in adult IgA vasculitis (Henoch-Schönlein purpura): updated picture from a French multicentre and retrospective series of 260 cases: reply
