Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg–Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain. He had eosinophilia, and electroneuromyography demonstrated sensorimotor polyneuropathy. His skin biopsy revealed necrotizing eosinophilic vasculitis and eosinophilic panniculitis. Although he had no respiratory symptoms or history of asthma, prominent pulmonary involvement was evident on thoracic MRI. After treatment, his complaints of pain improved but mild neuropathy persisted. After 4 years of follow-up, he had minimal hypoesthesia in his right hand but had not experienced any relapses. This case highlights the fact that in cases suspected of EGPA, even without respiratory symptoms or asthma, detailed imaging should be performed for a definitive diagnosis. In addition, mild neurological findings may persist despite treatment in EGPA. The relevant literature on EGPA, with specific reference to pediatric cases, is reviewed.
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The case was diagnosed and followed up by SY. SY conceived and planned the presented case report. GOY and SY wrote the manuscript with support from ZET and NÇD. Pathologic evaluation was performed by NÇD. Literature data were provided by SY, GOY and ZET. The manuscript was edited for proper English language by Gozen Translation and Editing services.
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Yener, G.O., Tekin, Z.E., Demirkan, N.Ç. et al. Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review. Rheumatol Int 38, 697–703 (2018). https://doi.org/10.1007/s00296-017-3884-1
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DOI: https://doi.org/10.1007/s00296-017-3884-1