Presentation
History of adult-onset asthma, presenting with more than a decade history of bilateral orbital/periorbital swelling and cervical/axillary lymphadenopathy.
Patient Data
Markedly enlarged extraocular muscles, abnormal soft tissue along the orbital walls, and abnormal soft tissue in the preseptal region bilaterally, consistent with orbital involvement by xanthogranuloma.
Additional involvement by abnormal soft tissue of the left infraorbital nerve canal and the bilateral pterygopalatine fossa.
Bilateral parotid nodules, which likely represent salivary involvement by xanthogranuloma.
Extensive bilateral parotid, submandibular, upper internal jugular and supraclavicular lymphadenopathy along with bilateral axillary and mediastinal lymphadenopathy represent patient's known inflammatory/infiltrative disorder.
Extensive soft tissue thickening in bilateral extraocular muscles and preseptal regions as well as in pterygopalatine regions, may be related to diffuse inflammatory or neoplastic disease process.
Extensive diffuse mucosal thickening or opacification of the paranasal sinuses is likely related to an infectious or inflammatory etiology.
Case Discussion
Eyelid skin biopsy showed xanthogranuloma. In the setting of adult onset asthma overlapping in time with these symptoms, yellowish discoloration of the eyelids, and xanthogranulomatous infiltration on histology, the final diagnosis was adult-onset asthma with periocular xanthogranulomas (AAPOX). Patients present with periorbital swelling, salivary gland enlargement, and lymphadenopathy with foamy histiocytes on biopsy.
This condition has been associated with IgG4 related disease spectrum 1. The histologic criteria for IgG4 related disease were not satisfied in this case's biopsy. However, the serum IgG4 was elevated at 849 mg/dL (upper limit of normal 86), supportive of but not conclusive for the diagnosis. Radiological features that are more likely in IgG4 related disease that other types of orbital inflammation include infraorbital nerve enlargement and concurrent paranasal sinus disease 2, as was seen in this case.
The patient was treated with rituxmab in accordance with prior reports 3, which led to a long-term decrease in periocular swelling/infiltration both clinically and radiologically but not complete resolution.
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