One-third of the patients with HS and IED did not have any autoimmune or inflammatory comorbidity that could explain the eye involvement. The potential association between HS and IED might be a manifestation of a common immune dysregulation phenomenon. Furthermore, the management of IED required an escalation of therapy to systemic immunosuppressive agents in 70% of patients with HS.

Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity of rare and complex immunemediated fibroinflammatory conditions that can affect any organ. The concomitance of IgG4 sclerosing sialadenitis and dacryoadenitis with rhinosinusitis is extremely rare. We report a case of IgG4 sclerosing sialadenitis and dacryoadenitis (Mikulicz's disease) diagnosed in a middle-aged African American man with a long-standing history of chronic rhinosinusitis who presented with progressively worsening bilateral salivary and lacrimal glands swelling. Imaging revealed pansinusitis, symmetric enlargement of the lacrimal glands, parotid glands, and submandibular glands. Serological IgG4 level was significantly elevated and the diagnosis of IgG4 sclerosing sialadenitis was confirmed by histopathology. A robust clinical response in the facial swelling and nasal manifestations was noted after the initiation of immunotherapy with corticosteroids.