Cutaneous polyarteritis nodosa in pediatric patients successfully treated with TNF‐α inhibitor and methotrexate: Case series and literature review

Abstract

Cutaneous polyarteritis nodosa (CPAN) is a rare necrotizing vasculitis affecting small‐ to medium‐sized arteries. Reported treatments include oral corticosteroids alone or in combination with non‐steroidal antiinflammatory drugs, intravenous immunoglobulins, cyclophosphamide, azathioprine, colchicine, or dapsone. However, some patients with CPAN do not respond to such treatments and continue to experience exacerbations over prolonged periods. This series provides support for the use of TNF‐α inhibitors in the treatment of recalcitrant CPAN in pediatric patients.

Methotrexate-Induced Leukocytoclastic Vasculitis

“…The clinical and histological findings, the temporal relationship between methotrexate intake and the onset of vasculitis, and the results of withdrawal and re-challenge tests suggest a causal relationship in our case [ 11 ]. Paradoxically, methotrexate has also been used for the treatment of LCV in patients with connective tissue disorders [ 2 - 4 ]. Though LCV is not life-threatening toxicity and most cases improve spontaneously, our child also developed transaminitis and deranged coagulation profile which were also attributed to methotrexate, and therefore her chemotherapy was withheld temporarily.…”

“…In children, methotrexate is prescribed for treatment of lymphoreticular malignancies like acute lymphoblastic leukemia (ALL) as well as various rheumatic diseases like juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), localized scleroderma, and psoriasis. Methotrexate is also used to treat vasculitis associated with rheumatic illnesses [2][3][4].…”

Localized Forms of Vasculitis

“…Mild cases presenting with nodular skin lesions and livedo reticularis can be managed with topical glucocorticoids and oral NSAIDs [55,69,73,74]. Dapsone can also be used [59,79]. Occasionally, lower doses of oral glucocorticoids (< 20mg/day of prednisone or equivalent) may be necessary to induce remission [55,59,69,71].…”

“…Rituximab has been suggested as an appropriate alternative to cyclophosphamide, particularly in patients with severe cutaneous arteritis presenting with ulcers or necrosis [80•]. Anti-TNF treatment, namely infliximab and etanercept, has also been successfully employed in six cases of refractory pediatric cutaneous arteritis [79]. Nevertheless, prospective studies are warranted.…”