Eosinophilic angiocentric fibrosis in paediatric IgG4-related disease.
Rupal Prasad, Parag Vijayvergia, Mahima Khatkar et al.
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Case in brief
A young girl presented with a 1.5-year history of progressive bilateral upper eyelid swelling and proptosis, more pronounced on the right. Despite partial improvement with oral prednisolone, symptoms recurred during tapering. MRI revealed right periorbital soft tissue thickening. Biopsy confirmed eosinophilic angiocentric fibrosis, leading to a diagnosis of possible organ-limited IgG4-related disease. Treatment with prednisolone and mycophenolate mofetil resulted in sustained improvement.
What made this case unusual
The case illustrates that pediatric IgG4-related disease can manifest as isolated orbital swelling despite normal serum IgG4 levels, emphasizing the importance of histopathological evaluation.
Diagnostic / clinical pearl
Histopathology is essential for diagnosing pediatric IgG4-related disease, especially when serum IgG4 levels are normal.