Cutaneous Vasculitis as Early Presentation of Eosinophilic Granulomatosis with Polyangiitis without Systemic Symptoms

Abstract

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a rare vasculitis in children. It commonly presents with respiratory symptoms, especially asthma, allergic rhinitis, and peripheral eosinophilia. Involvement of other systems, such as renal and cardiac, may carry a poor prognosis. Anti-neutrophil cytoplasmic antibodies have been found less frequently in children with this condition. We report a case of a 15-year-old male who presented with indurated and pruritic papules on both legs and peripheral eosinophilia without other system involvement. Histopathological findings from a skin biopsy were suggestive of eosinophilic granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibodies were negative.

Benralizumab: A novel treatment for the cutaneous features of paediatric eosinophilic granulomatosis with polyangiitis (pEGPA)

“…The most common findings are tender subcutaneous nodules, palpable purpura, petechiae and polymorphous erythematous eruptions 1,3,6 . Indurated hyperpigmented plaques, as seen in our patient, are atypical, having been described only once before in the literature 7 …”

“…1,3,6 Indurated hyperpigmented plaques, as seen in our patient, are atypical, having been described only once before in the literature. 7 Systemic glucocorticoids remain the mainstay of treatment, with additional immunomodulatory therapies, including methotrexate, azathioprine and cyclophosphamide to be considered depending on disease severity, specific organ involvement and treatment response. For less severe cases of EGPA, including where symptoms are limited to the respiratory system and skin, an anti-IL-5 agent may be preferred, such as the monoclonal antibodies mepolizumab and reslizumab, or the IL-5 receptor antibody benralizumab.…”

ANCA-Positive Small-Vessel Vasculitis Following SARS-CoV-2 Vaccination—A Systematic Review

“…Prior studies have strongly associated asthma with EGPA, as a crucial prodromal syndrome often manifesting many years before the active vasculitis phase. In patients with persistent asthma that is difficult to control, consideration should be given to the possibility of EGPA diagnosis [ 48 , 49 , 50 ]. According to ACR/EULAR criteria from 2022, clinical and laboratory criteria for EGPA assign obstructive airway disease and nasal polyps, respectively, +3 points each, and blood eosinophil count /L for +5 points, which strongly correlates with symptoms of asthma.…”