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Manifestations of skull base IgG4‐related disease: A multi‐institutional study

The Laryngoscope Oct 24, 2020

Marinelli JP, Marvisi C, Vaglio A, et al. - Given that IgG4‐related disease (IgG4‐RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor‐like lesions of the head and neck, researchers sought to identify the cranial base manifestations of IgG4‐RD. Eleven patients have been identified at a median age at presentation of 58 years. Cranial neuropathies in the abducens, trigeminal, and facial nerves were frequently found. According to findings, IgG4‐RD nonspecifically presents as a rare cause of the skull base mass. Local diagnostic biopsies are often needed with no concomitant systemic disease. Adequate tissue specimens are complicated by densely fibrotic cranial base lesions, often in close proximity to critical neurovascular structures. In most patients, primary medical therapy with corticosteroids and rituximab is successful.

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