IgG4-Related Disease: A Diagnostic Dilemma for 20 Years
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Updated: Nov 9, 2026

A Protocol for Rapid Post-mortem Cell Culture of Diffuse Intrinsic Pontine Glioma (DIPG)
Published on: March 7, 2017
Alexandra Murray1, Marlene Wijaya1, Jennifer Kim2,3
1Department of Dermatology, Westmead Hospital, Westmead, New South Wales, Australia.
Abstract:
We report a case of IgG4-related disease in a 65-year-old man who remained undiagnosed for two decades. His disease initially manifested as periorbital oedema, followed by right cheek swelling, lymphadenopathy and a widespread pruritic eruption of skin-coloured and pale erythematous papules. IgG4 was only considered after a stroke protocol MRI of the brain demonstrated an incidental finding of characteristic orbital manifestations. This case highlights the diagnostic complexity of IgG4-related disease and emphasises the importance of recognising its varied presentations to facilitate early diagnosis and management.
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