Adult-onset asthma and periocular xantogranuloma associated with IgG4-related disease: Report of a case

Adult-onset asthma and periocular xanthogranuloma (AAPOX) is a rare xanthogranulomatous disorder that may overlap with IgG4-related disease (IgG4-RD). We describe a 49-year-old woman with bilateral eyelid ptosis and yellowish subcutaneous nodules associated with mild asthma. Orbital MRI showed ill-defined inflammatory tissue in the superior extraconal fat, and biopsy revealed xanthogranulomatous inflammation with numerous IgG4-positive plasma cells. Systemic imaging revealed pancreatic and salivary gland involvement consistent with IgG4-RD. Corticosteroid therapy followed by rituximab achieved complete clinical and radiologic remission and normalization of serum IgG4 and IgE levels. This case illustrates the overlap between AAPOX and IgG4-RD and supports their possible coexistence within a common pathophysiologic spectrum.