IgG4-related disease presenting as otogenic skull base osteomyelitis

Abstract

IgG4-related disease (IgG4-RD) is an emerging clinical disease entity characterized by tumefactive lesions at multiple sites with a dense lymphoplasmacytic infiltrate rich in IgG4 + plasma cells.Although almost any organ can be affected, IgG4-RD is most likely to involve the submandibular, lacrimal, or parotid glands in the head and neck region. However, skull base involvement presenting as otogenic skull base osteomyelitis (SBO) is rare. We encountered a 70-year-old male with IgG4-RD presenting primarily with severe otalgia and otorrhea. He had uncontrolled diabetes mellitus and showed clinical manifestations of otogenic SBO. Tissue immunostaining revealed typical features of increased IgG4-positive plasma cells, and hematological examination showed elevated serum IgG4 concentrations. Treatment with corticosteroids significantly improved wellbeing and partially resolved the lesion based on computed tomography (CT) scan.

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

“…Some skull base locations of IgG4-RD documented in the literature include the meninges, sphenoid bone, sphenoid and maxillary sinus, orbit, clivus, internal auditory canal, brain parenchyma, infratemporal fossa, pterygopalatine fossa, suprasellar region, and temporal bone (9)(10)(11)(12)(13). The most recent guidelines from the American College of Rheumatologists and European League Against Rheumatism Guidelines (5) have shifted toward a point-based system where IgG4-RD diagnosis is based on a spectrum of classic features and absence of other plausible diagnoses.…”

Manifestaciones otológicas de la enfermedad relacionada con IgG4

“…Otras formas de presentación infrecuentes fueron parálisis facial periférica 13,20,27,31,34 habitualmente por infiltración pseudotumoral (Imagen 4), otorrea 19,28,40,43 por sobreinfección de una perforación timpánica o formación de granulomas inflamatorios, parestesias faciales (por afectación del V par), nódulos 25,44 auriculares, mastoiditis recurrente, acúfeno pulsátil o fístula retroauricular 15 .…”

Differential diagnosis of IgG4-related pancreatitis and pancreatic cancer by MRI features and its correlation with serum IgG4 level

“…The results suggested there was a difference in DDS, PD perforation sign, the proportion of main PD truncation, and the ratio of main PD diameter/pancreatic parenchymal width between the two groups (P < 0.05). DDS, truncation of the main PD at the lesion, and atrophy of the pancreatic parenchyma are widely considered highly suspicious findings for PC, and PD perforation sign, irregular dilatation of the main PD, and calcification of the pancreatic parenchyma are considered characteristic features of IgG4-related AIP (23,24). It revealed there was a distinction in the MRI features between IgG4-related AIP and PC.…”