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Eosinophilic granulomatosis with polyangiitis associated with subcutaneous nodules in a 10‐year‐old girl

Characterized by an eosinophilic necrotizing immune response in in the dermis, eosinophilic granulomatosis with polyangiitis (EGPA; formerly referred to as Churg-Strauss syndrome) is a vasculitis affecting small to medium-sized vessels [1]. Its exact pathogenesis is unknown. Antinuclear cytoplasmic antibodies (ANCA) are found in 30–38 % of cases [2]. While pediatric cases of eosinophilic granulomatosis with polyangiitis are extremely rare and usually ANCA-negative, they are frequently associated with bronchial asthma [3]. We report the case of a 10-year-old girl whose primary symptoms included painless subcutaneous nodules. Following histological, radiological, and serological workup, we diagnosed eosinophilic granulomatosis with polyangiitis. The patient presented with small nodules that had initially developed after a sojourn in the Dominican Republic. Apart from allergic bronchial asthma, she was healthy and in good general condition. The clinical exam revealed small, firm, mobile subcutaneous nodules on the arms and scalp, measuring up to 1 cm. There was also a partially indurated erythematous plaque on the back of the right foot, extending to its inner border. The differential blood count showed up to 31 % eosinophils. Subsequent serological workup for parasitosis (Ascaris lumbricoides, Dirofilaria immitis, Strongyloides spp., Toxocara spp., Trichinella spp., Trichinella spiralis, Cysticercus spp.) performed at the Bernhard Nocht Institute for Tropical Medicine in Hamburg yielded unremarkable findings. Immunofluorescence microscopy showed neither cytoplasmic nor perinuclear antinuclear cytoplasmic antibodies (c-ANCA and p-ANCA). Abdominal ultrasound and echocardiography were also normal. A CT scan of the thorax showed diffuse patchy consolidations in both lungs (Figure 1). Subsequent dermal and subcutaneous biopsy of one of the scalp nodules showed partially calcified necrosis and an accumulation of eosinophilic granulocytes in the form of eosinophilic granulomas (Figure 2). Given the local and systemic eosinophilia, a history of allergic bronchial asthma, and typical pulmonary infiltrates on CT, our patient fulfilled enough criteria to establish the diagnosis of eosinophilic granulomatosis with polyangiitis. Treatment was initiated following a pediatric rheumatology consult. The patient initially received methylprednisolone pulse therapy for 3 days, followed by a maintenance dose of 1 mg/kg, which was subsequently tapered. In addition, she was started on immunosuppressive therapy with methotrexate (12.5 mg SQ per week). The aforementioned combined regimen of corticosteroids and methotrexate resulted in marked regression of the disseminated subcutaneous nodules, and the lesion on the foot healed with postinflammatory hyperpigmentation. There was also a rapid drop in blood eosinophils. Figure 1 Computed tomography of the thorax showing patchy consolidations.