Two cases of igg4 related disease in paediatric and young patients with ocular and ear canal involvement

Abstract

IgG4 related disease is a newly recognised entity, more cases being reported, as pathologists and medical practitioners across the world are becoming more aware of this disease. It is an immune-mediated disease, that can appear with multiple organ involvement, as tumor-like swelling, mimicking other neoplasms like lymphomas, which must be carefully excluded. If untreated, the disease can lead to fibrosis and irreversible organ damage. Hence, early recognition of IgG4 related disease is important to avoid permanent organ dysfunction. IgG4 mostly has been described in adults, hence it is generally less known among the paediatricians. We report two cases – one is of an 11 year old male child with superior orbital swelling, clinically suspected to be an inflammatory lesion or a lymphoma. The other case is a 20 year old male, presented as small polyps in the left external auditory canal, these did not bleed on touch and covered the tympanic membrane.