The relation between malocclusion and nasal deformities

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ. In this case, a cerebral cavernous malformation (CCM) was incidentally detected by magnetic resonance imaging (MRI) performed to assess the progressive enlargement of the bilateral lacrimal glands. CCM caused a cerebral hemorrhage 7 days post-diagnosis.A 41-year-old man with a past medical history of significant bronchial asthma presented to our hospital with a 3-month history of a changing facial configuration. MRI had revealed a considerable enlargement of the bilateral lacrimal glands and a nodule surrounded by T2 and fluid-attenuated inversion recovery hyperintensity area in the left frontal lobe. He was planning to admit himself to our hospital 7 days after the initial MRI. Computed tomography on admission revealed a cerebral hemorrhage around the nodule. According to him, he had experienced a sudden headache and slight difficulty in speaking 2 days before admission. Neurological examination on admission revealed a slight facial motor weakness on the right side. Blood tests revealed a remarkable elevation of IgG:2623.9 and IgG4:1420.9. Tumor resection was performed 5 days after admission. Histopathological diagnosis was a cavernous malformation. Immunofluorescence staining revealed few IgG4-positive plasma cells. His neurological symptoms gradually disappeared. Parotid gland biopsy revealed numerous IgG4-positive cells, confirming the diagnosis of IgG4-RD. Prednisolone was prescribed, and MRI at 2 months post-operation revealed the normalization of the enlarged lacrimal and parotid glands.The effect of IgG4-RD on CCM remains unknown. The simultaneous worsening of IgG4-RD and hemorrhage from CCM in our case strongly suggests an association between IgG4-RD and CCM, and we consider cytokines to be the key factors in this association. Theoretically, increased levels of certain serum cytokines, such as IL-5, in patients with IgG4-RD could affect CCM. Underlying disorders should be considered when a progressive and aggressive CCM is detected.